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dzip1l  (Proteintech)


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    Structured Review

    Proteintech dzip1l
    Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens <t>DZIP1/DZIP1L</t> and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to
    Dzip1l, supplied by Proteintech, used in various techniques. Bioz Stars score: 93/100, based on 31 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/dzip1l/product/Proteintech
    Average 93 stars, based on 31 article reviews
    dzip1l - by Bioz Stars, 2026-03
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    Images

    1) Product Images from "The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers."

    Article Title: The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers.

    Journal: Advanced science (Weinheim, Baden-Wurttemberg, Germany)

    doi: 10.1002/advs.202308820

    Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens DZIP1/DZIP1L and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to
    Figure Legend Snippet: Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens DZIP1/DZIP1L and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to

    Techniques Used: Sequencing

    Figure 6. The precise localization of DZIP1L in human RPE cells. Spatial localization of DZIP1L was examined using a combination of Ultrastructure expansion microscopy (ExM) and confocal microscopy. As our DZIP1L polyclonal antibody is derived from rabbits, and most of our antibodies for TF and TZ markers are also polyclonal antibodies from rabbit, co-labeling them with antibodies is not feasible. To assess their co-localization, we employed transgenic FLAG-DZIP1L and utilized a mouse monoclonal FLAG antibody (IgG1 isotype) to label DZIP1L. While cilia were marked using anti-Ac-tubulin, a mouse monoclonal antibody with IgG2b isotype. A) FLAG-DZIP1L was localized beneath the classical TZ component CEP290 in both ciliated and non-
    Figure Legend Snippet: Figure 6. The precise localization of DZIP1L in human RPE cells. Spatial localization of DZIP1L was examined using a combination of Ultrastructure expansion microscopy (ExM) and confocal microscopy. As our DZIP1L polyclonal antibody is derived from rabbits, and most of our antibodies for TF and TZ markers are also polyclonal antibodies from rabbit, co-labeling them with antibodies is not feasible. To assess their co-localization, we employed transgenic FLAG-DZIP1L and utilized a mouse monoclonal FLAG antibody (IgG1 isotype) to label DZIP1L. While cilia were marked using anti-Ac-tubulin, a mouse monoclonal antibody with IgG2b isotype. A) FLAG-DZIP1L was localized beneath the classical TZ component CEP290 in both ciliated and non-

    Techniques Used: Microscopy, Confocal Microscopy, Derivative Assay, Labeling, Transgenic Assay

    Figure 7. Conserved role of DZIP1L-ANKRD26 module in cilia gating in human RPE cells. A) The genetic interaction between DZIP1L and ANKRD26 in cilia formation is conserved in mammalian cells. The knockouts of either DZIP1L or ANKRD26 led to a moderate decrease in ciliation ratio. However, when both DZIP1L and ANKRD26 were knocked out, cilia formation was nearly completely blocked. The ciliation ratio was shown in the lower panel. Data are presented as mean ± SEM. n > 300 cells from three independent experiments. Significant differences were determined by two tailed t-test analysis. Scale bars: 5μm. B) Ciliary entry of the IFT component IFT140 was compromised in double mutants of DZIP1L and ANKRD26, compared to either of
    Figure Legend Snippet: Figure 7. Conserved role of DZIP1L-ANKRD26 module in cilia gating in human RPE cells. A) The genetic interaction between DZIP1L and ANKRD26 in cilia formation is conserved in mammalian cells. The knockouts of either DZIP1L or ANKRD26 led to a moderate decrease in ciliation ratio. However, when both DZIP1L and ANKRD26 were knocked out, cilia formation was nearly completely blocked. The ciliation ratio was shown in the lower panel. Data are presented as mean ± SEM. n > 300 cells from three independent experiments. Significant differences were determined by two tailed t-test analysis. Scale bars: 5μm. B) Ciliary entry of the IFT component IFT140 was compromised in double mutants of DZIP1L and ANKRD26, compared to either of

    Techniques Used: Two Tailed Test



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    Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens <t>DZIP1/DZIP1L</t> and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to
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    Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens <t>DZIP1/DZIP1L</t> and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to
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    Pedigrees of the families. A : Family 1 . The two affected children (V:3 and V:6) are both homozygous for the <t>DZIP1L</t> sequence variant: c.216C > G; p.(Cys72Trp). a : Ultrasound scan of the left kidney in V:3 and b : Ultrasound scan of the left kidney in V:6. B . Family 2. The proband (III:3) is homozygous for the same sequence variant as identified in family 1 : c.216C > G; p.(Cys72Trp). C. Family 3 . The proband (VI:2) is homozygous for the DZIP1L sequence variant: c.193T > C; p.(Cys65Arg). Filled symbols indicate homozygous individuals, and half-filled symbols indicate heterozygous relatives. a : MR-scan of the kidneys from VI:2 showing enlarged kidneys with multiple cysts. b : Histological sections from the right kidney in HE staining (VI:2): Cortical and medulla areas with cysts. Subcapsular, a small area shows a more normal structure. For details, see text. Arrows indicate the probands
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    Image Search Results


    Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens DZIP1/DZIP1L and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to

    Journal: Advanced science (Weinheim, Baden-Wurttemberg, Germany)

    Article Title: The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers.

    doi: 10.1002/advs.202308820

    Figure Lengend Snippet: Figure 1. C. elegans DZIP-1 is an evolutionarily conserved basal body protein. A) The sequence alignment of two evolutionarily conserved domain (DZIP- like domain and the C2H2 zine finger domain) within C. elegans DZIP-1, H. sapiens DZIP1/DZIP1L and M. musculus DZIP1/DZIP1L. The Dzip-like_N domain (red line) is the most conserved domain in DZIP1/DZIP1L proteins. The ZnF_C2H2 domain is indicated by an orange line. And the highly conserved region is enclosed in the purple box. Asterisks are used to indicate the residues that, when mutated, can cause human ARPKD. B) DZIP-1 localizes to the ciliary base in C. elegans. The schematic diagram of phasmid cilia is depicted on the left, GFP-tagged DZIP-1 specifically localizes to

    Article Snippet: Protein localization was confirmed by immunofluorescence imaging. gRNA sequence was as follows: DZIP1L gRNA#1, 5′- TGATCATCTTGCGACGCCGG-3′; DZIP1L gRNA#2, 5′- CAGTCCATGCTATCATGGCG-3′; ANKRD26 gRNA#1, 5′-GGGTAGCTCACAATCCTCTG-3′; ANKRD26 gRNA#2, 5′- AAATTCTTGTAACTAGAGTG-3′; Antibodies: The following primary antibodies were used in this study: ANKRD26 (rabbit, GeneTex, GTX128255, 1:500), DZIP1L (rabbit, Proteintech, 17474-1-AP, 1:500), FBF1 (rabbit, Proteintech, 11531-1-AP, 1:500), ARL13B (rabbit, Proteintech, 17711-1-AP, 1:500), KIAA0586/TALPID3 (rabbit, Proteintech, 24421-1-AP, 1:500), SCLT1 (rabbit, Proteintech, 14875- 1-AP, 1:500), CEP164 (rabbit, Proteintech, 22227-1-AP, 1:500), CEP89 (rabbit, Abcam, ab204410, 1:500), ODF2 (rabbit, Abcam, ab43840, 1:500), ODF2 (mouse, Abnova, H00004957-M01, 1:500), γ-tublin (mouse, Sigma-Aldrich, T6557, 1:1,000), FAM92A (rabbit, Proteintech, 24803-1- AP, 1:500), CBY1 (rabbit, Proteintech, 12239-1-AP, 1:500), GFP(mouse, Roche, 11814460001,1:200), IFT140 (rabbit, Proteintech, 17460-1-AP, 1:500), GT335(mouse, adipogen life science, A40251903), GLI3 (AF3690, R&D Systems), PC2 (Baltimore Polycystic Kidney Disease (PKD) Research and Clinical Core Center), Ac-tubulin (mouse, sigma, T7451, 1:300), Flag (mouse, sigma, F1804, 1:300), CEP290 (rabbit, abcam, ab84870).

    Techniques: Sequencing

    Figure 6. The precise localization of DZIP1L in human RPE cells. Spatial localization of DZIP1L was examined using a combination of Ultrastructure expansion microscopy (ExM) and confocal microscopy. As our DZIP1L polyclonal antibody is derived from rabbits, and most of our antibodies for TF and TZ markers are also polyclonal antibodies from rabbit, co-labeling them with antibodies is not feasible. To assess their co-localization, we employed transgenic FLAG-DZIP1L and utilized a mouse monoclonal FLAG antibody (IgG1 isotype) to label DZIP1L. While cilia were marked using anti-Ac-tubulin, a mouse monoclonal antibody with IgG2b isotype. A) FLAG-DZIP1L was localized beneath the classical TZ component CEP290 in both ciliated and non-

    Journal: Advanced science (Weinheim, Baden-Wurttemberg, Germany)

    Article Title: The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers.

    doi: 10.1002/advs.202308820

    Figure Lengend Snippet: Figure 6. The precise localization of DZIP1L in human RPE cells. Spatial localization of DZIP1L was examined using a combination of Ultrastructure expansion microscopy (ExM) and confocal microscopy. As our DZIP1L polyclonal antibody is derived from rabbits, and most of our antibodies for TF and TZ markers are also polyclonal antibodies from rabbit, co-labeling them with antibodies is not feasible. To assess their co-localization, we employed transgenic FLAG-DZIP1L and utilized a mouse monoclonal FLAG antibody (IgG1 isotype) to label DZIP1L. While cilia were marked using anti-Ac-tubulin, a mouse monoclonal antibody with IgG2b isotype. A) FLAG-DZIP1L was localized beneath the classical TZ component CEP290 in both ciliated and non-

    Article Snippet: Protein localization was confirmed by immunofluorescence imaging. gRNA sequence was as follows: DZIP1L gRNA#1, 5′- TGATCATCTTGCGACGCCGG-3′; DZIP1L gRNA#2, 5′- CAGTCCATGCTATCATGGCG-3′; ANKRD26 gRNA#1, 5′-GGGTAGCTCACAATCCTCTG-3′; ANKRD26 gRNA#2, 5′- AAATTCTTGTAACTAGAGTG-3′; Antibodies: The following primary antibodies were used in this study: ANKRD26 (rabbit, GeneTex, GTX128255, 1:500), DZIP1L (rabbit, Proteintech, 17474-1-AP, 1:500), FBF1 (rabbit, Proteintech, 11531-1-AP, 1:500), ARL13B (rabbit, Proteintech, 17711-1-AP, 1:500), KIAA0586/TALPID3 (rabbit, Proteintech, 24421-1-AP, 1:500), SCLT1 (rabbit, Proteintech, 14875- 1-AP, 1:500), CEP164 (rabbit, Proteintech, 22227-1-AP, 1:500), CEP89 (rabbit, Abcam, ab204410, 1:500), ODF2 (rabbit, Abcam, ab43840, 1:500), ODF2 (mouse, Abnova, H00004957-M01, 1:500), γ-tublin (mouse, Sigma-Aldrich, T6557, 1:1,000), FAM92A (rabbit, Proteintech, 24803-1- AP, 1:500), CBY1 (rabbit, Proteintech, 12239-1-AP, 1:500), GFP(mouse, Roche, 11814460001,1:200), IFT140 (rabbit, Proteintech, 17460-1-AP, 1:500), GT335(mouse, adipogen life science, A40251903), GLI3 (AF3690, R&D Systems), PC2 (Baltimore Polycystic Kidney Disease (PKD) Research and Clinical Core Center), Ac-tubulin (mouse, sigma, T7451, 1:300), Flag (mouse, sigma, F1804, 1:300), CEP290 (rabbit, abcam, ab84870).

    Techniques: Microscopy, Confocal Microscopy, Derivative Assay, Labeling, Transgenic Assay

    Figure 7. Conserved role of DZIP1L-ANKRD26 module in cilia gating in human RPE cells. A) The genetic interaction between DZIP1L and ANKRD26 in cilia formation is conserved in mammalian cells. The knockouts of either DZIP1L or ANKRD26 led to a moderate decrease in ciliation ratio. However, when both DZIP1L and ANKRD26 were knocked out, cilia formation was nearly completely blocked. The ciliation ratio was shown in the lower panel. Data are presented as mean ± SEM. n > 300 cells from three independent experiments. Significant differences were determined by two tailed t-test analysis. Scale bars: 5μm. B) Ciliary entry of the IFT component IFT140 was compromised in double mutants of DZIP1L and ANKRD26, compared to either of

    Journal: Advanced science (Weinheim, Baden-Wurttemberg, Germany)

    Article Title: The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers.

    doi: 10.1002/advs.202308820

    Figure Lengend Snippet: Figure 7. Conserved role of DZIP1L-ANKRD26 module in cilia gating in human RPE cells. A) The genetic interaction between DZIP1L and ANKRD26 in cilia formation is conserved in mammalian cells. The knockouts of either DZIP1L or ANKRD26 led to a moderate decrease in ciliation ratio. However, when both DZIP1L and ANKRD26 were knocked out, cilia formation was nearly completely blocked. The ciliation ratio was shown in the lower panel. Data are presented as mean ± SEM. n > 300 cells from three independent experiments. Significant differences were determined by two tailed t-test analysis. Scale bars: 5μm. B) Ciliary entry of the IFT component IFT140 was compromised in double mutants of DZIP1L and ANKRD26, compared to either of

    Article Snippet: Protein localization was confirmed by immunofluorescence imaging. gRNA sequence was as follows: DZIP1L gRNA#1, 5′- TGATCATCTTGCGACGCCGG-3′; DZIP1L gRNA#2, 5′- CAGTCCATGCTATCATGGCG-3′; ANKRD26 gRNA#1, 5′-GGGTAGCTCACAATCCTCTG-3′; ANKRD26 gRNA#2, 5′- AAATTCTTGTAACTAGAGTG-3′; Antibodies: The following primary antibodies were used in this study: ANKRD26 (rabbit, GeneTex, GTX128255, 1:500), DZIP1L (rabbit, Proteintech, 17474-1-AP, 1:500), FBF1 (rabbit, Proteintech, 11531-1-AP, 1:500), ARL13B (rabbit, Proteintech, 17711-1-AP, 1:500), KIAA0586/TALPID3 (rabbit, Proteintech, 24421-1-AP, 1:500), SCLT1 (rabbit, Proteintech, 14875- 1-AP, 1:500), CEP164 (rabbit, Proteintech, 22227-1-AP, 1:500), CEP89 (rabbit, Abcam, ab204410, 1:500), ODF2 (rabbit, Abcam, ab43840, 1:500), ODF2 (mouse, Abnova, H00004957-M01, 1:500), γ-tublin (mouse, Sigma-Aldrich, T6557, 1:1,000), FAM92A (rabbit, Proteintech, 24803-1- AP, 1:500), CBY1 (rabbit, Proteintech, 12239-1-AP, 1:500), GFP(mouse, Roche, 11814460001,1:200), IFT140 (rabbit, Proteintech, 17460-1-AP, 1:500), GT335(mouse, adipogen life science, A40251903), GLI3 (AF3690, R&D Systems), PC2 (Baltimore Polycystic Kidney Disease (PKD) Research and Clinical Core Center), Ac-tubulin (mouse, sigma, T7451, 1:300), Flag (mouse, sigma, F1804, 1:300), CEP290 (rabbit, abcam, ab84870).

    Techniques: Two Tailed Test

    Pedigrees of the families. A : Family 1 . The two affected children (V:3 and V:6) are both homozygous for the DZIP1L sequence variant: c.216C > G; p.(Cys72Trp). a : Ultrasound scan of the left kidney in V:3 and b : Ultrasound scan of the left kidney in V:6. B . Family 2. The proband (III:3) is homozygous for the same sequence variant as identified in family 1 : c.216C > G; p.(Cys72Trp). C. Family 3 . The proband (VI:2) is homozygous for the DZIP1L sequence variant: c.193T > C; p.(Cys65Arg). Filled symbols indicate homozygous individuals, and half-filled symbols indicate heterozygous relatives. a : MR-scan of the kidneys from VI:2 showing enlarged kidneys with multiple cysts. b : Histological sections from the right kidney in HE staining (VI:2): Cortical and medulla areas with cysts. Subcapsular, a small area shows a more normal structure. For details, see text. Arrows indicate the probands

    Journal: Pediatric Nephrology (Berlin, Germany)

    Article Title: Detection of DZIP1L mutations by whole-exome sequencing in consanguineous families with polycystic kidney disease

    doi: 10.1007/s00467-022-05441-4

    Figure Lengend Snippet: Pedigrees of the families. A : Family 1 . The two affected children (V:3 and V:6) are both homozygous for the DZIP1L sequence variant: c.216C > G; p.(Cys72Trp). a : Ultrasound scan of the left kidney in V:3 and b : Ultrasound scan of the left kidney in V:6. B . Family 2. The proband (III:3) is homozygous for the same sequence variant as identified in family 1 : c.216C > G; p.(Cys72Trp). C. Family 3 . The proband (VI:2) is homozygous for the DZIP1L sequence variant: c.193T > C; p.(Cys65Arg). Filled symbols indicate homozygous individuals, and half-filled symbols indicate heterozygous relatives. a : MR-scan of the kidneys from VI:2 showing enlarged kidneys with multiple cysts. b : Histological sections from the right kidney in HE staining (VI:2): Cortical and medulla areas with cysts. Subcapsular, a small area shows a more normal structure. For details, see text. Arrows indicate the probands

    Article Snippet: The human DZIP1L (NM_173543) cDNA clone was purchased from Origene.

    Techniques: Sequencing, Variant Assay, Staining

    A HEK293 cells transiently transfected with GFP-DZIP1L WT, Cys72Trp and mGFP were labeled with an anti-DZIP1L antibody. The anti-DZIP1L antibody only labeled cells that were transfected with the DZIP1L constructs. Scale bar: 20 µm. B Normal adult human kidney samples were stained with the same anti-DZIP1L antibody. Arrows indicate strong punctuate staining observed in select cells. Arrowheads indicate weaker and more diffuse staining, mainly found in collecting duct cells. C Double staining with anti-DZIP1L (red) and AQP2 (green) shows that the strong punctuate staining is not restricted to AQP2 expressing cells in the collecting system

    Journal: Pediatric Nephrology (Berlin, Germany)

    Article Title: Detection of DZIP1L mutations by whole-exome sequencing in consanguineous families with polycystic kidney disease

    doi: 10.1007/s00467-022-05441-4

    Figure Lengend Snippet: A HEK293 cells transiently transfected with GFP-DZIP1L WT, Cys72Trp and mGFP were labeled with an anti-DZIP1L antibody. The anti-DZIP1L antibody only labeled cells that were transfected with the DZIP1L constructs. Scale bar: 20 µm. B Normal adult human kidney samples were stained with the same anti-DZIP1L antibody. Arrows indicate strong punctuate staining observed in select cells. Arrowheads indicate weaker and more diffuse staining, mainly found in collecting duct cells. C Double staining with anti-DZIP1L (red) and AQP2 (green) shows that the strong punctuate staining is not restricted to AQP2 expressing cells in the collecting system

    Article Snippet: The human DZIP1L (NM_173543) cDNA clone was purchased from Origene.

    Techniques: Transfection, Labeling, Construct, Staining, Double Staining, Expressing

    The six DZIP1L sequence variants reported to date. All detected in homozygous form

    Journal: Pediatric Nephrology (Berlin, Germany)

    Article Title: Detection of DZIP1L mutations by whole-exome sequencing in consanguineous families with polycystic kidney disease

    doi: 10.1007/s00467-022-05441-4

    Figure Lengend Snippet: The six DZIP1L sequence variants reported to date. All detected in homozygous form

    Article Snippet: The human DZIP1L (NM_173543) cDNA clone was purchased from Origene.

    Techniques: Sequencing