alg5 Search Results


alg5  (Novus Biologicals)
93
Novus Biologicals alg5
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Alg5, supplied by Novus Biologicals, used in various techniques. Bioz Stars score: 93/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/alg5/product/Novus Biologicals
Average 93 stars, based on 1 article reviews
alg5 - by Bioz Stars, 2026-03
93/100 stars
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ab34710  (Proteintech)
93
Proteintech ab34710
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Ab34710, supplied by Proteintech, used in various techniques. Bioz Stars score: 93/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/ab34710/product/Proteintech
Average 93 stars, based on 1 article reviews
ab34710 - by Bioz Stars, 2026-03
93/100 stars
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alg5 open reading frame orf  (OriGene)
93
OriGene alg5 open reading frame orf
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Alg5 Open Reading Frame Orf, supplied by OriGene, used in various techniques. Bioz Stars score: 93/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/alg5 open reading frame orf/product/OriGene
Average 93 stars, based on 1 article reviews
alg5 open reading frame orf - by Bioz Stars, 2026-03
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janus (chi/alg)5 capsule micromotors  (CH Instruments)
90
CH Instruments janus (chi/alg)5 capsule micromotors
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Janus (Chi/Alg)5 Capsule Micromotors, supplied by CH Instruments, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/janus (chi/alg)5 capsule micromotors/product/CH Instruments
Average 90 stars, based on 1 article reviews
janus (chi/alg)5 capsule micromotors - by Bioz Stars, 2026-03
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pdl2-alg5  (Mobitec Inc)
90
Mobitec Inc pdl2-alg5
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Pdl2 Alg5, supplied by Mobitec Inc, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/pdl2-alg5/product/Mobitec Inc
Average 90 stars, based on 1 article reviews
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biochar-doped sodium alginate alg5-bc1  (Biocomposites Inc)
90
Biocomposites Inc biochar-doped sodium alginate alg5-bc1
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Biochar Doped Sodium Alginate Alg5 Bc1, supplied by Biocomposites Inc, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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Average 90 stars, based on 1 article reviews
biochar-doped sodium alginate alg5-bc1 - by Bioz Stars, 2026-03
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alg5 biomarker  (Molecular Biosciences Inc)
90
Molecular Biosciences Inc alg5 biomarker
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Alg5 Biomarker, supplied by Molecular Biosciences Inc, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/alg5 biomarker/product/Molecular Biosciences Inc
Average 90 stars, based on 1 article reviews
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bait vectors, pccw-suc, pncw, pai-alg5  (Dualsystems Biotech)
90
Dualsystems Biotech bait vectors, pccw-suc, pncw, pai-alg5
Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant <t>R79W-ALG5</t> (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.
Bait Vectors, Pccw Suc, Pncw, Pai Alg5, supplied by Dualsystems Biotech, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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alg5 gt 2  (Glycos Biotechnologies Inc)
90
Glycos Biotechnologies Inc alg5 gt 2
Properties of the components of N-linked glycosylation systems
Alg5 Gt 2, supplied by Glycos Biotechnologies Inc, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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primary antibody alg5 a175693  (ABclonal Biotechnology)
90
ABclonal Biotechnology primary antibody alg5 a175693
Properties of the components of N-linked glycosylation systems
Primary Antibody Alg5 A175693, supplied by ABclonal Biotechnology, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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go-alg/5-fu  (NanoCarrier Co)
90
NanoCarrier Co go-alg/5-fu
Properties of the components of N-linked glycosylation systems
Go Alg/5 Fu, supplied by NanoCarrier Co, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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Image Search Results


Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant R79W-ALG5 (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Family F350. (a) The F350 pedigree shows a family with autosomal dominant atypical polycystic kidney disease and a slow decline in glomerular filtration rate (GFR). Clinically affected individuals (black symbols) had bilateral kidney cysts, reduced GFR below 60 ml/min per 1.73 m 2 or nonenlarged kidneys with nephronophthisis-like histology. Gray indicates young carriers of the variant with no established clinical diagnosis yet. White indicates clinically unaffected individuals. Out of the 28 individuals evaluated in this family, 18 were determined as genetically affected individuals with the heterozygous missense variant R79W-ALG5 (+/−), whereas 10 individuals were genetically unaffected (−/−). A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. (Bottom - Left) Radiologic imaging of genetically affected members of F350 (b–j). Yellow arrows denote kidney cysts, whereas red arrows indicate cysts in the liver. (b and c) T2-weighted magnetic resonance imaging (MRI) of 65-year-old patient II.1 demonstrates bilateral multiple scattered kidney and liver cysts. (d) At age 64, II.3 underwent an MRI scan that revealed nonenlarged cystic kidney and high-burden hepatic simple cysts (>20 cysts, with the largest measuring 11.6 cm in the right lobe - red arrows;). He underwent liver cysts fenestration at 70 years of age as the result of ongoing pain. An MRI of the liver performed at the age of 71 years (e) confirms the presence of severe polycystic liver disease. (f and g) A CT scan of II. 4's kidneys and liver at age 59 showed multiple subcentimeter-sized kidney cysts and a solitary liver cyst in segment 2. Similar to II.3, a 72-year-old MRI scan of II.12 reveals scattered kidney cysts (1 cyst is displayed in h) and (i) numerous liver cysts, whereas (j) a CT scan of II.13 reveals only a few kidney cysts. (Bottom - Right) (k, o, and s) Kidney biopsy slides staining of 3 genetically affected individuals from F350. At 52, 51, and 54 years of age, subjects II.2, II.10, and II.11, respectively, underwent clinically indicated kidney biopsies; their corresponding estimated glomerular filtration rate at the time of the biopsies were 37, 32, and 54 ml/min per 1.73 m 2 . Hematoxylin and eosin (H&E) stained section showing a central cystically dilated tubule in all patients marked with an asterisk (∗). (Images l, p, and t) Also, a variable degree of inflammatory infiltrate within the tubulointerstitial compartment has been identified–using H&E stain. (m, q, and u) Masson-Trichrome and (n, r, and v) Silver stained sections illustrating variable degrees of glomerulosclerosis, interstitial fibrosis, and tubular atrophy. All biopsies captured at 10× magnification with scale bars represent 100 μm. CT, computed tomography; MRI, magnetic resonance imaging.

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Filtration, Variant Assay, Imaging, Magnetic Resonance Imaging, Computed Tomography, Staining

Family F200. (a) The pedigree of F200 shows 5 clinically affected members (black symbols). Gray indicates clinically unclear individuals and white indicates clinically unaffected individuals. A (+/−) sign indicates the presence of the heterozygous missense variant R79W-ALG5 and a (−/−) sign indicates the presence of the wild-type WT-ALG5. A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. Out of the 10 individuals evaluated in this family, 5 were determined as genetically affected individuals with a heterozygous missense variant R79W-ALG5 (+/−), whereas the remaining members were genetically unaffected (−/−). (b and c) CT scan of II.14 at the age of 86 years revealed nonenlarged cystic kidneys (yellow arrows) and severe polycystic liver disease (red arrows). (d and e) Subject II.5 at the age of 67 years, who does not harbor the ALG5 variant, has a few kidney and scattered liver cysts with estimated glomerular filtration rate 57 ml/min per 1.73 m 2 . Disease-causing variants in PKD1, PKD2, PRKCSH, ALG9, ALG8, DNAJB11, SEC63, and GANAB , among other monogenic kidney disorders, were analyzed and none were identified, using exome sequencing. Subjects II.4, II.11 and II.13 enjoy a preserved kidney function; eGFR 66 ml/min for subject II.4, 80 ml/min for subject II.11 and 69 ml/min for subject II.13 at age of 76, 77, and 73 years, respectively. None of the subjects had any kidney cysts: Subject II.4 (at age 76 years via CT scan with contrast with slice thickness of 1 mm), subject II.11 (at age 69 years via CT abdomen and pelvis imaging with contrast; slice thickness of 1.5 mm) and subject II.13 (at age 70 years via US abdomen). Subjects II.4 and II.11 have been found to have small simple liver cysts, estimated to be less than approximately 5 to 10 in count. CT, computed tomography; US, ultrasound.

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Family F200. (a) The pedigree of F200 shows 5 clinically affected members (black symbols). Gray indicates clinically unclear individuals and white indicates clinically unaffected individuals. A (+/−) sign indicates the presence of the heterozygous missense variant R79W-ALG5 and a (−/−) sign indicates the presence of the wild-type WT-ALG5. A (+) sign indicates the presence of the R79W-ALG5 variant, and a (−) sign indicates the presence of the wild-type WT-ALG5 in a genotyped individual. Out of the 10 individuals evaluated in this family, 5 were determined as genetically affected individuals with a heterozygous missense variant R79W-ALG5 (+/−), whereas the remaining members were genetically unaffected (−/−). (b and c) CT scan of II.14 at the age of 86 years revealed nonenlarged cystic kidneys (yellow arrows) and severe polycystic liver disease (red arrows). (d and e) Subject II.5 at the age of 67 years, who does not harbor the ALG5 variant, has a few kidney and scattered liver cysts with estimated glomerular filtration rate 57 ml/min per 1.73 m 2 . Disease-causing variants in PKD1, PKD2, PRKCSH, ALG9, ALG8, DNAJB11, SEC63, and GANAB , among other monogenic kidney disorders, were analyzed and none were identified, using exome sequencing. Subjects II.4, II.11 and II.13 enjoy a preserved kidney function; eGFR 66 ml/min for subject II.4, 80 ml/min for subject II.11 and 69 ml/min for subject II.13 at age of 76, 77, and 73 years, respectively. None of the subjects had any kidney cysts: Subject II.4 (at age 76 years via CT scan with contrast with slice thickness of 1 mm), subject II.11 (at age 69 years via CT abdomen and pelvis imaging with contrast; slice thickness of 1.5 mm) and subject II.13 (at age 70 years via US abdomen). Subjects II.4 and II.11 have been found to have small simple liver cysts, estimated to be less than approximately 5 to 10 in count. CT, computed tomography; US, ultrasound.

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Variant Assay, Computed Tomography, Filtration, Sequencing, Imaging

In Silico structural mapping of missense variants in ALG5. (a) Diagram of the ALG5 sequence. Novel pathogenic variant identified in this study is shown in red. Previously reported dominant pathogenic variants associated with late-onset ADPKD are shown in blue. (b) Computational structural model of human dolichyl-phosphate beta-glucosyltransferase activity generated by AlphaFold (AF-Q9Y673-F1). Reaction products, dolichyl-phosphate (Dol-P) and Uridine 5'-diphospho (UDP)-alpha-D-glucose (UDP-Glc), are placed at the enzyme active site according to data from the crystal structure of archeal dolichyl-phosphate mannose synthase (see methods). Flexible region of the A-loop governing a dolichyl-phosphate beta-glucosyltransferase activity is highlighted in green. Mutated arginine residues previously reported (R208H, R212H) and the variant from this study (R79W) are shown as red sticks. The last 2 models represent mutants carrying premature stop codons. Missing parts of ALG5 protein and neopeptide in Gln235Valfs∗21 are highlighted by red and magenta, respectively. (c) Amino acid conservation across mutated segments of ALG5 in mammals. Asterisks (∗) indicate amino acid residues that are absolutely conserved and a colon (:) indicates residues with strong conservation across species. (d) Population allele frequency of R79W-ALG5 variant in GnomAD v4.0.0 database and computational prediction by Mendelian Clinically Applicable Pathogenicity Score (M-CAP) of the pathogenicity of missense R79W-ALG5 variant. ADPKD, autosomal dominant polycystic kidney disease.

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: In Silico structural mapping of missense variants in ALG5. (a) Diagram of the ALG5 sequence. Novel pathogenic variant identified in this study is shown in red. Previously reported dominant pathogenic variants associated with late-onset ADPKD are shown in blue. (b) Computational structural model of human dolichyl-phosphate beta-glucosyltransferase activity generated by AlphaFold (AF-Q9Y673-F1). Reaction products, dolichyl-phosphate (Dol-P) and Uridine 5'-diphospho (UDP)-alpha-D-glucose (UDP-Glc), are placed at the enzyme active site according to data from the crystal structure of archeal dolichyl-phosphate mannose synthase (see methods). Flexible region of the A-loop governing a dolichyl-phosphate beta-glucosyltransferase activity is highlighted in green. Mutated arginine residues previously reported (R208H, R212H) and the variant from this study (R79W) are shown as red sticks. The last 2 models represent mutants carrying premature stop codons. Missing parts of ALG5 protein and neopeptide in Gln235Valfs∗21 are highlighted by red and magenta, respectively. (c) Amino acid conservation across mutated segments of ALG5 in mammals. Asterisks (∗) indicate amino acid residues that are absolutely conserved and a colon (:) indicates residues with strong conservation across species. (d) Population allele frequency of R79W-ALG5 variant in GnomAD v4.0.0 database and computational prediction by Mendelian Clinically Applicable Pathogenicity Score (M-CAP) of the pathogenicity of missense R79W-ALG5 variant. ADPKD, autosomal dominant polycystic kidney disease.

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: In Silico, Sequencing, Variant Assay, Activity Assay, Generated

Kidney function in genetically affected individuals. The most recent estimated glomerular filtration rate (eGFR) (ml/min per 1.73 m 2 ) versus age in individuals affected with the missense mutation p.R79W in ALG5. Solid triangles indicate genetically affected individuals. Hollow circles indicate genetically unaffected individuals; none of whom developed chronic kidney disease, except on individual F200-II.9 (individual with a history of multiple comorbidities, namely atrial fibrillation, osteoarthritis, recurrent cystitis, microscopic hematuria, and active tobacco use; please refer to the manuscript for details). Most affected individuals developed chronic kidney disease stage 3 (horizontal line) after the age of 50 years (vertical line). Five individuals of varying ages had reached end-stage kidney disease, as indicated by an eGFR value of 5 ml/min per 1.73 m 2 (Y-axis).

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Kidney function in genetically affected individuals. The most recent estimated glomerular filtration rate (eGFR) (ml/min per 1.73 m 2 ) versus age in individuals affected with the missense mutation p.R79W in ALG5. Solid triangles indicate genetically affected individuals. Hollow circles indicate genetically unaffected individuals; none of whom developed chronic kidney disease, except on individual F200-II.9 (individual with a history of multiple comorbidities, namely atrial fibrillation, osteoarthritis, recurrent cystitis, microscopic hematuria, and active tobacco use; please refer to the manuscript for details). Most affected individuals developed chronic kidney disease stage 3 (horizontal line) after the age of 50 years (vertical line). Five individuals of varying ages had reached end-stage kidney disease, as indicated by an eGFR value of 5 ml/min per 1.73 m 2 (Y-axis).

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Filtration, Mutagenesis

Clinical and radiological characterization of the 23 affected members in whom the novel pathogenic R79W-ALG5 variant was confirmed

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Clinical and radiological characterization of the 23 affected members in whom the novel pathogenic R79W-ALG5 variant was confirmed

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Variant Assay, Imaging

Detection of ALG5 in kidney biopsy. (a–d) ALG5 staining in genetically affected individual F350_II.2 with the missense variant p.R79W in ALG5 and (e–h) in a control subject. ALG5 protein was detected in all segments of nephron-glomerulus (G), proximal tubules (PT), distal tubules (DT), collecting ducts (CD), and thick ascending loop of Henle cells (TALH). In the affected subject was observed the coarsely granular intracytoplasmic staining of ALG5 compared to the finely granular and less intense staining in the control subject. (i–x) Intracellular localization of ALG5 in kidney biopsy. In affected kidney biopsy, parallel staining of ALG5 with Protein disulfide isomerase (PDI) (i in detail j), a marker of endoplasmic reticulum (ER), and with 58K Golgi-protein (q in detail r) demonstrate expected albeit less intense localization of the ALG5 in the ER (k in detail l) and pathologic localization in the Golgi (s in detail s). In the control kidney, parallel staining with PDI (m in detail n) and 58K Golgi protein (u in detail v) demonstrated localization of ALG5 in ER (o in detail p) but not in the Golgi (w in detail x). In the lower right corner of each image (5K, 5O, 5S, 5W) is Pearson coefficient ± SEM to show mean degree of colocalization between individual compartment markers and ALG5 in all analyzed tubules. The degree of ALG5 colocalization with selected markers is presented as the fluorescent signal overlap coefficient values that range from 0 to 1. The corresponding lookup table displays the resulting overlap coefficient values as the pseudo-color scale.

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Detection of ALG5 in kidney biopsy. (a–d) ALG5 staining in genetically affected individual F350_II.2 with the missense variant p.R79W in ALG5 and (e–h) in a control subject. ALG5 protein was detected in all segments of nephron-glomerulus (G), proximal tubules (PT), distal tubules (DT), collecting ducts (CD), and thick ascending loop of Henle cells (TALH). In the affected subject was observed the coarsely granular intracytoplasmic staining of ALG5 compared to the finely granular and less intense staining in the control subject. (i–x) Intracellular localization of ALG5 in kidney biopsy. In affected kidney biopsy, parallel staining of ALG5 with Protein disulfide isomerase (PDI) (i in detail j), a marker of endoplasmic reticulum (ER), and with 58K Golgi-protein (q in detail r) demonstrate expected albeit less intense localization of the ALG5 in the ER (k in detail l) and pathologic localization in the Golgi (s in detail s). In the control kidney, parallel staining with PDI (m in detail n) and 58K Golgi protein (u in detail v) demonstrated localization of ALG5 in ER (o in detail p) but not in the Golgi (w in detail x). In the lower right corner of each image (5K, 5O, 5S, 5W) is Pearson coefficient ± SEM to show mean degree of colocalization between individual compartment markers and ALG5 in all analyzed tubules. The degree of ALG5 colocalization with selected markers is presented as the fluorescent signal overlap coefficient values that range from 0 to 1. The corresponding lookup table displays the resulting overlap coefficient values as the pseudo-color scale.

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Staining, Variant Assay, Control, Marker

Uromodulin (UMOD) investigations. (a) Plasma concentration of UMOD decreases with reduced estimated glomerular filtration rate (ml/min per 1.73 m 2 ) in individuals affected with the missense variant p.R79W in ALG5, individuals with UMOD pathogenic variants and individuals with unspecified CKD. The decrease in UMOD concentration is slower and less expressive in cases with the ALG5 variant than in cases with UMOD mutations. A best-fit line is included for each group (b) Semiquantitative and qualitative detection of urinary UMOD. Western blot of spot urine samples from 10 healthy controls, 1 patient with a canonical variant in UMOD (ADTKD- UMOD ), and 6 genetically affected individuals from family F350 and family F200 showed a reduced amount of urinary UMOD in all investigated patients compared to healthy controls. No abnormality in electrophoretic mobility of residual UMOD suggestive of abnormal N-linked glycosylation was observed in patients. The sample volume was normalized to urinary creatinine level. Intracellular localization of UMOD in patients and control kidney biopsy. To elucidate the cause of the reduction of urinary excretion and the plasma level of UMOD, we analyzed its intracellular localization in kidney biopsy from the patient and control. In affected kidney biopsy, parallel staining of uromodulin (UMOD) with Protein disulfide isomerase (PDI), a marker of endoplasmic reticulum (ER), and with pan-Cadherin, (c in detail d a marker of the plasma membrane (PM)) demonstrate localization of the uromodulin in both, (d) partly in the ER (34%) and (f) partly on PM (66%). In the control kidney, (g in detail h) parallel staining of UMOD with PDI and pan-Cadherin demonstrated (i) localization of UMOD on PM (92%) (j) but not in the ER. In the upper right corner of each image (6K, 6F, 6I, 6J) is the Pearson coefficient ± SEM to show mean degree of colocalization between individual compartment markers and ALG5 in all analyzed tubules. The degree of UMOD colocalization with selected markers is demonstrated by the fluorescent signal overlap coefficient values ranging from 0 to 1. The resulting overlap coefficient values are presented as the pseudo-color, which scale is shown in the corresponding lookup table.

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Uromodulin (UMOD) investigations. (a) Plasma concentration of UMOD decreases with reduced estimated glomerular filtration rate (ml/min per 1.73 m 2 ) in individuals affected with the missense variant p.R79W in ALG5, individuals with UMOD pathogenic variants and individuals with unspecified CKD. The decrease in UMOD concentration is slower and less expressive in cases with the ALG5 variant than in cases with UMOD mutations. A best-fit line is included for each group (b) Semiquantitative and qualitative detection of urinary UMOD. Western blot of spot urine samples from 10 healthy controls, 1 patient with a canonical variant in UMOD (ADTKD- UMOD ), and 6 genetically affected individuals from family F350 and family F200 showed a reduced amount of urinary UMOD in all investigated patients compared to healthy controls. No abnormality in electrophoretic mobility of residual UMOD suggestive of abnormal N-linked glycosylation was observed in patients. The sample volume was normalized to urinary creatinine level. Intracellular localization of UMOD in patients and control kidney biopsy. To elucidate the cause of the reduction of urinary excretion and the plasma level of UMOD, we analyzed its intracellular localization in kidney biopsy from the patient and control. In affected kidney biopsy, parallel staining of uromodulin (UMOD) with Protein disulfide isomerase (PDI), a marker of endoplasmic reticulum (ER), and with pan-Cadherin, (c in detail d a marker of the plasma membrane (PM)) demonstrate localization of the uromodulin in both, (d) partly in the ER (34%) and (f) partly on PM (66%). In the control kidney, (g in detail h) parallel staining of UMOD with PDI and pan-Cadherin demonstrated (i) localization of UMOD on PM (92%) (j) but not in the ER. In the upper right corner of each image (6K, 6F, 6I, 6J) is the Pearson coefficient ± SEM to show mean degree of colocalization between individual compartment markers and ALG5 in all analyzed tubules. The degree of UMOD colocalization with selected markers is demonstrated by the fluorescent signal overlap coefficient values ranging from 0 to 1. The resulting overlap coefficient values are presented as the pseudo-color, which scale is shown in the corresponding lookup table.

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Concentration Assay, Filtration, Variant Assay, Western Blot, Control, Staining, Marker, Membrane

Hypothetical pathogenetic mechanisms of monoallelic ALG5 variants. Upper left cartoon: ALG5 is an N-glycosylated protein that localizes in the endoplasmic reticulum (ER) membrane. Its biosynthesis proceeds through cotranslational translocation into ER and posttranslational modifications, including sequential maturation of N-linked oligosaccharides and folding in the ER, ERGIC, and Golgi apparatus. Fully maturated and properly folded ALG5 is finally retrieved back to the ER. Lower left cartoon: ALG5 catalyzes on the cytoplasmic face of ER membrane synthesis of dolichyl beta-D-glucosyl phosphate (Dol-P-Glc) from dolichyl phosphate and Uridine 5'-diphospho (UDP)-alpha-D-glucose. Dol-P-Glc is subsequently translocated by flippase into the ER lumen, where it provides glucose (Glc) residues for the sequential building of the growing lipid linked-oligosaccharides (LLO) by ALG6, ALG8, and ALG10. The resulting oligosaccharide glycan core consists typically of Man 9 Glc 3 GlcNAc 2 ; The Man 9 Glc 3 GlcNAc 2 oligosaccharide is transferred to the asparagine (N) residue of the polypeptide chain by an oligosaccharyltransferase complex OSTA that glycosylates nascent polypeptides traversing the translocon or OSTB that glycosylates proteins in the ER lumen. Then, folding of N-linked glycoproteins proceeds via subsequent removal of 3 Glc residues by glucosidases (Gluc I, Gluc IIα, and Gluc IIβ) and one mannose (Man) residue by ER mannosidase (MAN1B1) with assistance of chaperones (calnexin, calreticulin). Properly folded and Man 8 GlcNAc 2 modified glycoproteins then traverse via COP II coated vesicles through ER-Golgi intermediate compartment (ERGIC) to the Golgi for final processing and sorting for transport to their eventual destinations. Upper right cartoon: the p.R79W variant affects the maturation, folding, and trafficking of the ALG5, leading to aberrant protein deposition within the Golgi apparatus and disturbance of Golgi homeostasis. Lower right cartoon: the p.R79W variant and ALG5 haploinsufficiency affects synthesis and structure of the LLO oligosaccharides with negative consequences on maturation, trafficking, and intracellular localization of other N-glycosylated proteins.

Journal: Kidney International Reports

Article Title: A Novel Monoallelic ALG5 Variant Causing Late-Onset ADPKD and Tubulointerstitial Fibrosis

doi: 10.1016/j.ekir.2024.04.031

Figure Lengend Snippet: Hypothetical pathogenetic mechanisms of monoallelic ALG5 variants. Upper left cartoon: ALG5 is an N-glycosylated protein that localizes in the endoplasmic reticulum (ER) membrane. Its biosynthesis proceeds through cotranslational translocation into ER and posttranslational modifications, including sequential maturation of N-linked oligosaccharides and folding in the ER, ERGIC, and Golgi apparatus. Fully maturated and properly folded ALG5 is finally retrieved back to the ER. Lower left cartoon: ALG5 catalyzes on the cytoplasmic face of ER membrane synthesis of dolichyl beta-D-glucosyl phosphate (Dol-P-Glc) from dolichyl phosphate and Uridine 5'-diphospho (UDP)-alpha-D-glucose. Dol-P-Glc is subsequently translocated by flippase into the ER lumen, where it provides glucose (Glc) residues for the sequential building of the growing lipid linked-oligosaccharides (LLO) by ALG6, ALG8, and ALG10. The resulting oligosaccharide glycan core consists typically of Man 9 Glc 3 GlcNAc 2 ; The Man 9 Glc 3 GlcNAc 2 oligosaccharide is transferred to the asparagine (N) residue of the polypeptide chain by an oligosaccharyltransferase complex OSTA that glycosylates nascent polypeptides traversing the translocon or OSTB that glycosylates proteins in the ER lumen. Then, folding of N-linked glycoproteins proceeds via subsequent removal of 3 Glc residues by glucosidases (Gluc I, Gluc IIα, and Gluc IIβ) and one mannose (Man) residue by ER mannosidase (MAN1B1) with assistance of chaperones (calnexin, calreticulin). Properly folded and Man 8 GlcNAc 2 modified glycoproteins then traverse via COP II coated vesicles through ER-Golgi intermediate compartment (ERGIC) to the Golgi for final processing and sorting for transport to their eventual destinations. Upper right cartoon: the p.R79W variant affects the maturation, folding, and trafficking of the ALG5, leading to aberrant protein deposition within the Golgi apparatus and disturbance of Golgi homeostasis. Lower right cartoon: the p.R79W variant and ALG5 haploinsufficiency affects synthesis and structure of the LLO oligosaccharides with negative consequences on maturation, trafficking, and intracellular localization of other N-glycosylated proteins.

Article Snippet: For parallel immunodetection of either ALG5, UMOD, or MUC1 with ER, ER-Golgi intermediate compartment, Golgi apparatus, or plasma membrane markers, the following antibodies were used: ALG5 with polyclonal rabbit anti-ALG5 antibody (Novus Biologicals) diluted 1:200; UMOD with polyclonal sheep anti-THP/UMOD antibody (Tamm-Horsfall Glycoprotein antibody, My BioSource) diluted 1:300; MUC1 with monoclonal Mouse antiHuman Epithelial Membrane Antigen antibody (Dako, Glostrup, Denmark) diluted 1 : 100; ER with monoclonal mouse anti-PDI antibody (ENZO Life Sciences) diluted 1:50 or rabbit anti-SEC61A (Abcam #14379] diluted 1 : 300; ER-Golgi intermediate compartment with monoclonal mouse anti-LMAN1 antibody (Thermo Fisher Scientific) diluted 1:100; Golgi apparatus with monoclonal mouse anti-58K antibody (Abcam) diluted 1:50 and plasma membrane with rabbit antipan Cadherin (Thermo Fisher Scientific) diluted 1:50 in 5% BSA in PBS.

Techniques: Membrane, Translocation Assay, Residue, Modification, Variant Assay

Properties of the components of N-linked glycosylation systems

Journal:

Article Title: AglC and AglK Are Involved in Biosynthesis and Attachment of Diacetylated Glucuronic Acid to the N-Glycan in Methanococcus voltae ▿

doi: 10.1128/JB.00885-08

Figure Lengend Snippet: Properties of the components of N-linked glycosylation systems

Article Snippet: Protein Function a TMD b CD c S. cerevisiae ( Eukaryota ) 1370470 Alg5 Gt 2 Glycos_transf_2 6325441 Dpm1 Gt 1 Glycos_transf_2 536653 Alg7 Gt 11 Glycos_transf_4 6321391/6319544 Alg13/ Alg14 Gt 2/2 Glyco_tran_28_C/ none 536377 Alg1 Gt 3 Glycos_transf_1 1322572 Alg2 Gt 4 Glycos_transf_1 1301907 Alg11 Gt 3 Glycos_transf_1 536015 Rft1 Flippase 12 Rft protein 586444 Alg3 Gt 10 ALG3 protein 1302235 Alg9 Gt 10 Glyco_transf_22 47678269 Alg12 Gt 13 Glyco_transf_22 1420090 Alg6 Gt 13 Alg6_Alg8 1420215 Alg8 Gt 14 Alg6_Alg8 1706435 Alg10 Gt 11 DIE2/ALG10 6321416 Stt3p Ot 14 STT3 C. jejuni ( Bacteria ) 5771412 PglC Gt 1 Bac_transf 5771411 PglA Gt 0 Glycos_transf_1 57166812 PglJ Gt 2 Glycos_transf_1 57166814 PglH Gt 2 Glycos_transf_1 57166813 PglI Gt 1 Glycos_transf_2 3413446 PglK Flippase 6 ABC_ATPase 5771410 PglB Ot 11 STT3 M. voltae ( Archaea ) 87045855 AlgH Gt 7 Glycos_transf_4 Unknown Unknown Gt Unknown Unknown 87045840 AglA Gt 1 Glycos_transf_1 Unknown Unknown Flippase Unknown Unknown 87045854 AglB Ot 13 STT3 M. voltae genes targeted 190336425 Mv990 Gt candidate 2 Glycos_transf_2 190336423 Mv991 Gt candidate 1 Glycos_transf_2 190403021 Mv891 Flippase candidate 11 Polysacc_synt Open in a separate window a Gt, glycosyl transferase; Ot, oligosaccharyl transferase. b TMDs were predicted by TMpred. c CDs are from the NCBI Conserved Domain Database.

Techniques: Glycoproteomics, Bacteria