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Proteintech primary antibodies include rabbit anti dnajc7
$A CRISPRi screen of molecular chaperones identifies <t>DNAJC7</t> as a modifier of polyQ aggregation. A , workflow of CRISPRi screen to identify molecular chaperone modifiers of polyQ aggregation in NLS-FRET-Q79 cells. Created with BioRender. B , volcano plot CRISPRi screen results, highlighting DNAJ protein and proteasomal subunit hits. C , flow cytometry plots of NLS-FRET-Q79 cells at 4 days of doxycycline with or without proteasomal inhibitor (carfilzomib). D and E , Western blot ( D ) and quantification ( E ) of DNAJC7 levels in NLS-FRET-Q79 cells expressing nontargeting control (NTC) sgRNAs or sgRNAs targeting DNAJC7. Each lane represents lysate from an independent well; bars and error bars indicate mean ± SD, and points represent n = 3 wells. Signal intensities were normalized to total protein and then to sgNTC#1. F , results of flow cytometry measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with sgNTC or sgDNAJC7. Data are presented as fold change relative to the mean of the sgNTCs for each independent experiment. Bars and error bars represent mean ± SD, and points represent n = 3 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). CRISPRi, CRISPR interference. NLS, nuclear localization signal; polyQ, polyglutamine; sgRNA, single guide RNA.$
Primary Antibodies Include Rabbit Anti Dnajc7, supplied by Proteintech, used in various techniques. Bioz Stars score: 93/100, based on 13 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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primary antibodies include rabbit anti dnajc7 - by Bioz Stars, 2026-05

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1) Product Images from "The Hsp40 cochaperone DNAJC7 regulates polyglutamine aggregation and exhibits context-dependent effects on polyglycine aggregation"

Article Title: The Hsp40 cochaperone DNAJC7 regulates polyglutamine aggregation and exhibits context-dependent effects on polyglycine aggregation

Journal: The Journal of Biological Chemistry

doi: 10.1016/j.jbc.2026.111292

$A CRISPRi screen of molecular chaperones identifies DNAJC7 as a modifier of polyQ aggregation. A , workflow of CRISPRi screen to identify molecular chaperone modifiers of polyQ aggregation in NLS-FRET-Q79 cells. Created with BioRender. B , volcano plot CRISPRi screen results, highlighting DNAJ protein and proteasomal subunit hits. C , flow cytometry plots of NLS-FRET-Q79 cells at 4 days of doxycycline with or without proteasomal inhibitor (carfilzomib). D and E , Western blot ( D ) and quantification ( E ) of DNAJC7 levels in NLS-FRET-Q79 cells expressing nontargeting control (NTC) sgRNAs or sgRNAs targeting DNAJC7. Each lane represents lysate from an independent well; bars and error bars indicate mean ± SD, and points represent n = 3 wells. Signal intensities were normalized to total protein and then to sgNTC#1. F , results of flow cytometry measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with sgNTC or sgDNAJC7. Data are presented as fold change relative to the mean of the sgNTCs for each independent experiment. Bars and error bars represent mean ± SD, and points represent n = 3 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). CRISPRi, CRISPR interference. NLS, nuclear localization signal; polyQ, polyglutamine; sgRNA, single guide RNA.$
Figure Legend Snippet: A CRISPRi screen of molecular chaperones identifies DNAJC7 as a modifier of polyQ aggregation. A , workflow of CRISPRi screen to identify molecular chaperone modifiers of polyQ aggregation in NLS-FRET-Q79 cells. Created with BioRender. B , volcano plot CRISPRi screen results, highlighting DNAJ protein and proteasomal subunit hits. C , flow cytometry plots of NLS-FRET-Q79 cells at 4 days of doxycycline with or without proteasomal inhibitor (carfilzomib). D and E , Western blot ( D ) and quantification ( E ) of DNAJC7 levels in NLS-FRET-Q79 cells expressing nontargeting control (NTC) sgRNAs or sgRNAs targeting DNAJC7. Each lane represents lysate from an independent well; bars and error bars indicate mean ± SD, and points represent n = 3 wells. Signal intensities were normalized to total protein and then to sgNTC#1. F , results of flow cytometry measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with sgNTC or sgDNAJC7. Data are presented as fold change relative to the mean of the sgNTCs for each independent experiment. Bars and error bars represent mean ± SD, and points represent n = 3 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). CRISPRi, CRISPR interference. NLS, nuclear localization signal; polyQ, polyglutamine; sgRNA, single guide RNA.

Techniques Used: Flow Cytometry, Western Blot, Expressing, Control, Transduction, CRISPR

$DNAJC7 suppresses mutant Huntingtin exon 1 (HTTex1) aggregation and colocalizes with inclusions. A , schematic of a lentiviral construct encoding a doxycycline-inducible eGFP-tagged fragment of HTTex1 containing 72 glutamines (GFP-HTTex1-Q72) used to generate a monoclonal HEK293T CRISPRi cell line. B , fluorescence micrograph of GFP-HTTex1-Q72 cells at 7 days of doxycycline treatment before and after treatment with Triton X-100. C , results of flow cytometry experiments measuring the fraction of total events containing detergent-resistant GFP + cells comparing those stably transduced with NTC or DNAJC7-targeting sgRNAs and normalized to the mean of NTCs. Data represent mean ± SD from n = 4 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). D , fluorescence micrographs of HEK293T cells 48 h after transient cotransfection of GFP-HTTex1-Q72 and either mTagBFP2 (BFP) alone or BFP-tagged DNAJC7. Arrows indicate cytoplasmic aggregates. E , flow cytometry plotting GFP-height versus GFP-width (pulse shape analysis) of HEK293T cells transfected with GFP-HTTex1-Q72 for 48 h. The detergent-resistant population of cells is designated as aggregate positive (Agg + ). F , results of flow cytometry experiments measuring Agg + cells in HEK293T cells 48 h after transient cotransfection with GFP-HTTex1-Q72 and either BFP or BFP-DNAJC7. Bars and error bars represent mean ± SD from n = 4 independent biological replicates (indicated by color), with each performed in technical triplicate. ∗ p < 0.05 by Student’s t test using mean values of technical replicates. The scale bars represent 10 μm. CRISPRi, CRISPR interference; eGFP, enhanced GFP; HEK293T, human embryonic kidney 293T cell line; NTC, nontargeting control; sgRNA, single guide RNA.$
Figure Legend Snippet: DNAJC7 suppresses mutant Huntingtin exon 1 (HTTex1) aggregation and colocalizes with inclusions. A , schematic of a lentiviral construct encoding a doxycycline-inducible eGFP-tagged fragment of HTTex1 containing 72 glutamines (GFP-HTTex1-Q72) used to generate a monoclonal HEK293T CRISPRi cell line. B , fluorescence micrograph of GFP-HTTex1-Q72 cells at 7 days of doxycycline treatment before and after treatment with Triton X-100. C , results of flow cytometry experiments measuring the fraction of total events containing detergent-resistant GFP + cells comparing those stably transduced with NTC or DNAJC7-targeting sgRNAs and normalized to the mean of NTCs. Data represent mean ± SD from n = 4 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). D , fluorescence micrographs of HEK293T cells 48 h after transient cotransfection of GFP-HTTex1-Q72 and either mTagBFP2 (BFP) alone or BFP-tagged DNAJC7. Arrows indicate cytoplasmic aggregates. E , flow cytometry plotting GFP-height versus GFP-width (pulse shape analysis) of HEK293T cells transfected with GFP-HTTex1-Q72 for 48 h. The detergent-resistant population of cells is designated as aggregate positive (Agg + ). F , results of flow cytometry experiments measuring Agg + cells in HEK293T cells 48 h after transient cotransfection with GFP-HTTex1-Q72 and either BFP or BFP-DNAJC7. Bars and error bars represent mean ± SD from n = 4 independent biological replicates (indicated by color), with each performed in technical triplicate. ∗ p < 0.05 by Student’s t test using mean values of technical replicates. The scale bars represent 10 μm. CRISPRi, CRISPR interference; eGFP, enhanced GFP; HEK293T, human embryonic kidney 293T cell line; NTC, nontargeting control; sgRNA, single guide RNA.

Techniques Used: Mutagenesis, Construct, Fluorescence, Flow Cytometry, Stable Transfection, Transduction, Cotransfection, Transfection, CRISPR, Control

$Chaperone CRISPRi screening reveals limited suppression of polyG aggregation despite effects by DNAJC7 overexpression. A , volcano plot showing results of a CRISPRi screen for molecular chaperone modifiers of polyG aggregation in the NLS-FRET-G100 cell line. B , pairwise comparison of gene scores between the polyG screen (from A ) and the polyQ screen ( B ). C , results of flow cytometry experiments measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with NTC or DNAJC7. Data represent mean ± SD from n = 3 independent experiments. D , representative fluorescence micrographs of HEK293T cells transiently cotransfected with NLS-mScarlet- and NLS-mNeonGreen-tagged uN2C-G100 together with either BFP or BFP-DNAJC7; only the mNeonGreen channel is shown. The scale bar represents 10 μm. E , flow cytometry quantification of the percentage of FRET-high cells relative to total cells expressing both mNeonGreen and mScarlet. Bars indicate mean ± SD from n = 3 independent experiments. ∗ p < 0.05 by Student’s t test. CRISPRi, CRISPR interference; HEK293T, human embryonic kidney 293T cell line; NLS, nuclear localization signal; NTC, nontargeting control; polyG, polyglucine; polyQ, polyglutamine; sgRNA, single guide RNA.$
Figure Legend Snippet: Chaperone CRISPRi screening reveals limited suppression of polyG aggregation despite effects by DNAJC7 overexpression. A , volcano plot showing results of a CRISPRi screen for molecular chaperone modifiers of polyG aggregation in the NLS-FRET-G100 cell line. B , pairwise comparison of gene scores between the polyG screen (from A ) and the polyQ screen ( B ). C , results of flow cytometry experiments measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with NTC or DNAJC7. Data represent mean ± SD from n = 3 independent experiments. D , representative fluorescence micrographs of HEK293T cells transiently cotransfected with NLS-mScarlet- and NLS-mNeonGreen-tagged uN2C-G100 together with either BFP or BFP-DNAJC7; only the mNeonGreen channel is shown. The scale bar represents 10 μm. E , flow cytometry quantification of the percentage of FRET-high cells relative to total cells expressing both mNeonGreen and mScarlet. Bars indicate mean ± SD from n = 3 independent experiments. ∗ p < 0.05 by Student’s t test. CRISPRi, CRISPR interference; HEK293T, human embryonic kidney 293T cell line; NLS, nuclear localization signal; NTC, nontargeting control; polyG, polyglucine; polyQ, polyglutamine; sgRNA, single guide RNA.

Techniques Used: Over Expression, Comparison, Flow Cytometry, Transduction, Fluorescence, Expressing, CRISPR, Control

Image Search Results

Knockdown of dnajc7 leads to impaired neurodevelopment through accelerated TDP-43 aggregation. a Schematic representation and representative image of optogenetically modified transgenic zebrafish Tg[mnr2b-hs:opTDP-43h] Tg[mnr2b-hs:EGFP-TDP-43z]. The opTDP-43h construct encodes a human TDP-43 anchoring optic module, cryptochrome (CRY2), and RFP. Under basal conditions, the red signal is weakly expressed in the nucleus. After blue light illumination for 48–96 h, a clear aggregation of TDP-43 is observed at perinuclear sites. EGFP-TDP-43z encodes zebrafish TDP-43, which is recruited to and aggregated at the perinuclear site upon light-induced aggregation of opTDP-43 (yellow foci indicated by white arrows). b (Left) Schematic representation of CRISPR-Cas9-mediated dnajc7 knockout in zebrafish. (Middle) Quantitative reverse transcription PCR analysis of dnajc7 expression levels in sg ctr and sg dnajc7 injected embryos at 6 dpf (days post-fertilization). (Right) Agarose gel image showing the T7 endonuclease I assay results for the sgRNAs targeting dnajc7 , confirming successful knockout. c (Left) Representative microscopic images showing TDP-43 aggregates in sg ctr and sg dnajc7 injected ALS zebrafish. (Right) Quantification of pixel count for TDP-43 aggregates in sg ctr (7 fields) and sg dnajc7 (8 fields) injected ALS zebrafish at 6 dpf. Data are presented as mean ± SD from one of three independent experiments. Statistical differences were calculated using the Mann–Whitney U-test. d Fraction of dead embryos in sg ctr and sg dnajc7 injected ALS zebrafish at 6 dpf. Data show the number of dead or survived embryos from one of three independent experiments. Differences were calculated using Fisher’s exact test. e (Left) Fraction of embryos with failed SB inflation in sg ctr and sg dnajc7 injected ALS zebrafish at 6 dpf. (Middle) Representative images of SB in the embryos. (Right) Quantitative reverse transcription PCR analysis of dnajc7 expression in the survived sg dnajc7 injected embryos at 6dpf. Embryos with SB inflation failure showed significantly lower dnajc7 mRNA expression than those with normal SB inflation. Data show mean ± SD from one of three independent experiments. Statistical differences were calculated using Fisher's exact test or Mann–Whitney U-test. SB, swim bladder; ALS, amyotrophic lateral sclerosis; TDP-43, TAR DNA-binding protein of 43 kDa

Journal: Acta Neuropathologica

Article Title: Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology

doi: 10.1007/s00401-025-02899-y

Figure Lengend Snippet: Knockdown of dnajc7 leads to impaired neurodevelopment through accelerated TDP-43 aggregation. a Schematic representation and representative image of optogenetically modified transgenic zebrafish Tg[mnr2b-hs:opTDP-43h] Tg[mnr2b-hs:EGFP-TDP-43z]. The opTDP-43h construct encodes a human TDP-43 anchoring optic module, cryptochrome (CRY2), and RFP. Under basal conditions, the red signal is weakly expressed in the nucleus. After blue light illumination for 48–96 h, a clear aggregation of TDP-43 is observed at perinuclear sites. EGFP-TDP-43z encodes zebrafish TDP-43, which is recruited to and aggregated at the perinuclear site upon light-induced aggregation of opTDP-43 (yellow foci indicated by white arrows). b (Left) Schematic representation of CRISPR-Cas9-mediated dnajc7 knockout in zebrafish. (Middle) Quantitative reverse transcription PCR analysis of dnajc7 expression levels in sg ctr and sg dnajc7 injected embryos at 6 dpf (days post-fertilization). (Right) Agarose gel image showing the T7 endonuclease I assay results for the sgRNAs targeting dnajc7 , confirming successful knockout. c (Left) Representative microscopic images showing TDP-43 aggregates in sg ctr and sg dnajc7 injected ALS zebrafish. (Right) Quantification of pixel count for TDP-43 aggregates in sg ctr (7 fields) and sg dnajc7 (8 fields) injected ALS zebrafish at 6 dpf. Data are presented as mean ± SD from one of three independent experiments. Statistical differences were calculated using the Mann–Whitney U-test. d Fraction of dead embryos in sg ctr and sg dnajc7 injected ALS zebrafish at 6 dpf. Data show the number of dead or survived embryos from one of three independent experiments. Differences were calculated using Fisher’s exact test. e (Left) Fraction of embryos with failed SB inflation in sg ctr and sg dnajc7 injected ALS zebrafish at 6 dpf. (Middle) Representative images of SB in the embryos. (Right) Quantitative reverse transcription PCR analysis of dnajc7 expression in the survived sg dnajc7 injected embryos at 6dpf. Embryos with SB inflation failure showed significantly lower dnajc7 mRNA expression than those with normal SB inflation. Data show mean ± SD from one of three independent experiments. Statistical differences were calculated using Fisher's exact test or Mann–Whitney U-test. SB, swim bladder; ALS, amyotrophic lateral sclerosis; TDP-43, TAR DNA-binding protein of 43 kDa

Article Snippet: The following probe sets were used: HSPA1A (Hs00359163_s1), HSPB1 (Hs00356629_g1), BAG2 (Hs00989341_m1), DNAJC7 (Hs00268602_m1), and GAPDH (Hs02786624_g1) as the endogenous control.

Techniques: Knockdown, Modification, Transgenic Assay, Construct, CRISPR, Knock-Out, Reverse Transcription, Expressing, Injection, Agarose Gel Electrophoresis, T7EI Assay, MANN-WHITNEY, Binding Assay

Identification of homozygous DNAJC7 variants in three patients with ALS. a The pedigree chart of a family with three ALS patients. The proband is indicated by an arrow. b Brain T1-weighted magnetic resonance images of the proband (IV-5), with red arrows showing characteristic atrophy of the bilateral frontal lobes. Scale bar: 50 mm. c Brain computed tomography images of the proband’s older sister (IV-4), with red arrows indicating atrophy of the bilateral frontal lobes. Scale bar: 50 mm. d Parametric multipoint LOD scores showing significant peaks on chromosomes 5 and 17, suggesting a genetic linkage. e Chromatograms confirming the homozygous c.518dupC variant in DNAJC7 in all three affected individuals. f Schematic representation of the DNAJC7 protein, with the ALS-associated variant located within the tetratricopeptide repeat domain. ALS, amyotrophic lateral sclerosis; LOD, logarithm of odds

Journal: Acta Neuropathologica

Article Title: Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology

doi: 10.1007/s00401-025-02899-y

Figure Lengend Snippet: Identification of homozygous DNAJC7 variants in three patients with ALS. a The pedigree chart of a family with three ALS patients. The proband is indicated by an arrow. b Brain T1-weighted magnetic resonance images of the proband (IV-5), with red arrows showing characteristic atrophy of the bilateral frontal lobes. Scale bar: 50 mm. c Brain computed tomography images of the proband’s older sister (IV-4), with red arrows indicating atrophy of the bilateral frontal lobes. Scale bar: 50 mm. d Parametric multipoint LOD scores showing significant peaks on chromosomes 5 and 17, suggesting a genetic linkage. e Chromatograms confirming the homozygous c.518dupC variant in DNAJC7 in all three affected individuals. f Schematic representation of the DNAJC7 protein, with the ALS-associated variant located within the tetratricopeptide repeat domain. ALS, amyotrophic lateral sclerosis; LOD, logarithm of odds

Techniques: Computed Tomography, Variant Assay

Phosphorylated TDP-43 pathology, Bunina bodies, and severe loss of the upper and lower motor neurons in the older brother (IV-1) with a homozygous c.518dupC frameshift variant in DNAJC7. a Lateral view of the left hemisphere, showing severe atrophy in the precentral gyrus (asterisks). Arrowhead indicates the top of the precentral gyrus, the width of which is extremely thin. b Severe neuronal loss with gliosis in all cortical layers of the primary motor cortex. Betz cells are nearly completely absent. H&E stain. Scale bar: 200 μm. c Primary motor cortex in a 68-year-old pathologically normal control case. Betz cells are present in layer V, and the structure of the cerebral cortex is spared. H&E stain. Scale bar: 200 μm. d Severe neuronal loss with gliosis in the hypoglossal nucleus. A remaining neuron shows shrinkage of the cell body. H&E stain. Scale bar: 50 μm. e Hypoglossal nucleus in a 51-year-old pathologically normal control case. Neither glial proliferation nor shrinkage of neurons is observed. H&E stain. Scale bar: 50 μm. f Severe degeneration in the pyramidal tract within the medulla oblongata, as visualized by Klüver–Barrera stain. Scale bar: 500 μm. g , h Severe loss of myelin ( g ) with microglial activation ( h ) in the lateral tract of the thoracic cord. The dorsal spinocerebellar tract does not exhibit these changes. g Klüver–Barrera stain, h Iba-1 immunohistochemistry. Scale bars: g 1 mm and h 500 μm. i Degeneration in the lateral tract of the lumbar cord, as shown by Klüver–Barrera stain. Scale bar: 1 mm. j Severe loss of anterior horn cells in the lumbar cord, visualized by Klüver–Barrera stain. Scale bar: 500 μm. k Severe gliosis with loss of neurons in the anterior horn of the lumbar cord. H&E stain. Scale bar: 50 μm. l The Spinal anterior horns in a 67-year-old pathologically normal control case. Neither loss of anterior horn cells nor gliosis is present. H&E stain. Scale bar: 50 μm. m Bunina bodies in the lumber anterior horn. H&E stain. Scale bar: 10 μm. n Bunina body in the lumbar anterior horn, identified by Cystatin C immunohistochemistry. Scale bar: 10 μm. o Phosphorylated TDP-43-positive NCIs in the upper layers of the primary motor cortex. Neurites are barely discernible. pS409/410-2 immunohistochemistry. Scale bar: 50 μm. p – s Various morphologies of TDP-43-positive NCIs in the lumbar anterior horns. Inclusions include p skein-like, q neurofibrillary change-like, r diffuse granular, and s round structures. pS409/410-2 immunohistochemistry. Scale bars: 10 μm. t Phosphorylated TDP-43-positive glial cytoplasmic inclusion in the pyramidal tract of the medulla oblongata. pS409/410-2 immunohistochemistry. Scale bar: 10 μm. c , e , l Control cases presented here lacked any neurodegenerative changes except for minimal NFTs with Braak stage I. H&E, hematoxylin–eosin; NCI, neuronal cytoplasmic inclusions; NFTs, neurofibrillary tangles; TDP-43, TAR DNA-binding protein of 43 kDa

Journal: Acta Neuropathologica

Article Title: Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology

doi: 10.1007/s00401-025-02899-y

Figure Lengend Snippet: Phosphorylated TDP-43 pathology, Bunina bodies, and severe loss of the upper and lower motor neurons in the older brother (IV-1) with a homozygous c.518dupC frameshift variant in DNAJC7. a Lateral view of the left hemisphere, showing severe atrophy in the precentral gyrus (asterisks). Arrowhead indicates the top of the precentral gyrus, the width of which is extremely thin. b Severe neuronal loss with gliosis in all cortical layers of the primary motor cortex. Betz cells are nearly completely absent. H&E stain. Scale bar: 200 μm. c Primary motor cortex in a 68-year-old pathologically normal control case. Betz cells are present in layer V, and the structure of the cerebral cortex is spared. H&E stain. Scale bar: 200 μm. d Severe neuronal loss with gliosis in the hypoglossal nucleus. A remaining neuron shows shrinkage of the cell body. H&E stain. Scale bar: 50 μm. e Hypoglossal nucleus in a 51-year-old pathologically normal control case. Neither glial proliferation nor shrinkage of neurons is observed. H&E stain. Scale bar: 50 μm. f Severe degeneration in the pyramidal tract within the medulla oblongata, as visualized by Klüver–Barrera stain. Scale bar: 500 μm. g , h Severe loss of myelin ( g ) with microglial activation ( h ) in the lateral tract of the thoracic cord. The dorsal spinocerebellar tract does not exhibit these changes. g Klüver–Barrera stain, h Iba-1 immunohistochemistry. Scale bars: g 1 mm and h 500 μm. i Degeneration in the lateral tract of the lumbar cord, as shown by Klüver–Barrera stain. Scale bar: 1 mm. j Severe loss of anterior horn cells in the lumbar cord, visualized by Klüver–Barrera stain. Scale bar: 500 μm. k Severe gliosis with loss of neurons in the anterior horn of the lumbar cord. H&E stain. Scale bar: 50 μm. l The Spinal anterior horns in a 67-year-old pathologically normal control case. Neither loss of anterior horn cells nor gliosis is present. H&E stain. Scale bar: 50 μm. m Bunina bodies in the lumber anterior horn. H&E stain. Scale bar: 10 μm. n Bunina body in the lumbar anterior horn, identified by Cystatin C immunohistochemistry. Scale bar: 10 μm. o Phosphorylated TDP-43-positive NCIs in the upper layers of the primary motor cortex. Neurites are barely discernible. pS409/410-2 immunohistochemistry. Scale bar: 50 μm. p – s Various morphologies of TDP-43-positive NCIs in the lumbar anterior horns. Inclusions include p skein-like, q neurofibrillary change-like, r diffuse granular, and s round structures. pS409/410-2 immunohistochemistry. Scale bars: 10 μm. t Phosphorylated TDP-43-positive glial cytoplasmic inclusion in the pyramidal tract of the medulla oblongata. pS409/410-2 immunohistochemistry. Scale bar: 10 μm. c , e , l Control cases presented here lacked any neurodegenerative changes except for minimal NFTs with Braak stage I. H&E, hematoxylin–eosin; NCI, neuronal cytoplasmic inclusions; NFTs, neurofibrillary tangles; TDP-43, TAR DNA-binding protein of 43 kDa

Techniques: Variant Assay, Staining, Control, Activation Assay, Immunohistochemistry, Binding Assay

$Reduced expression of DNAJC7 protein in the motor cortex of FALS cases with the DNAJC7 variant. a Brain sarkosyl-insoluble fractions from patients with FALS (IV-1, IV-4) and patients with FTLD of various subtypes were subjected to immunoblot analysis using a phosphorylated-TDP43 (Ser409/410) antibody. The immunoblot revealed a distinct pattern of phosphorylated TDP-43, including a prominent band at approximately 50 kDa corresponding to full-length TDP-43, and C-terminal fragments of approximately 23–25 kDa, consistent with the type B pattern previously described . b Representative immunoblot showing DNAJC7 protein levels in motor cortex lysates from two FALS patients (IV-1 and IV-4), two SALS patients, and four controls. β-actin was used as the internal loading control. The asterisk (*) indicates the specific band corresponding to DNAJC7. Notably, DNAJC7 protein was barely detectable in the two FALS cases. c Immunohistochemical staining of brain sections from a patient with FALS (IV-1), a patient with SALS, and a control individual using an anti- DNAJC7 antibody. Scale bar: 50 µm. d Representative images of double -fluorescent staining of SALS brain sections with anti-TDP-43 and anti- DNAJC7 antibodies. Scale bar: 20 µm. SALS, sporadic amyotrophic lateral sclerosis; FALS, familial amyotrophic lateral sclerosis; FTLD, frontotemporal lobar degeneration; TDP-43, TAR DNA-binding protein of 43 kDa$

Journal: Acta Neuropathologica

Article Title: Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology

doi: 10.1007/s00401-025-02899-y

Figure Lengend Snippet: Reduced expression of DNAJC7 protein in the motor cortex of FALS cases with the DNAJC7 variant. a Brain sarkosyl-insoluble fractions from patients with FALS (IV-1, IV-4) and patients with FTLD of various subtypes were subjected to immunoblot analysis using a phosphorylated-TDP43 (Ser409/410) antibody. The immunoblot revealed a distinct pattern of phosphorylated TDP-43, including a prominent band at approximately 50 kDa corresponding to full-length TDP-43, and C-terminal fragments of approximately 23–25 kDa, consistent with the type B pattern previously described . b Representative immunoblot showing DNAJC7 protein levels in motor cortex lysates from two FALS patients (IV-1 and IV-4), two SALS patients, and four controls. β-actin was used as the internal loading control. The asterisk (*) indicates the specific band corresponding to DNAJC7. Notably, DNAJC7 protein was barely detectable in the two FALS cases. c Immunohistochemical staining of brain sections from a patient with FALS (IV-1), a patient with SALS, and a control individual using an anti- DNAJC7 antibody. Scale bar: 50 µm. d Representative images of double -fluorescent staining of SALS brain sections with anti-TDP-43 and anti- DNAJC7 antibodies. Scale bar: 20 µm. SALS, sporadic amyotrophic lateral sclerosis; FALS, familial amyotrophic lateral sclerosis; FTLD, frontotemporal lobar degeneration; TDP-43, TAR DNA-binding protein of 43 kDa

Techniques: Expressing, Variant Assay, Western Blot, Control, Immunohistochemical staining, Staining, Binding Assay

RNA-seq analysis revealed that DNAJC7 expression was decreased in the motor cortex of the FALS cases with the DNAJC7 variant. a Bar graph showing the number of genes that were upregulated or downregulated in patients with FALS (IV-1, IV-4) compared to normal controls. b Waterfall plot depicting the top ten upregulated and downregulated genes in patients with FALS. c Volcano plot illustrating the statistical significance against the fold change for gene expression in patients with FALS. FALS, familial amyotrophic lateral sclerosis; RNA-seq, RNA sequencing

Journal: Acta Neuropathologica

Article Title: Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology

doi: 10.1007/s00401-025-02899-y

Figure Lengend Snippet: RNA-seq analysis revealed that DNAJC7 expression was decreased in the motor cortex of the FALS cases with the DNAJC7 variant. a Bar graph showing the number of genes that were upregulated or downregulated in patients with FALS (IV-1, IV-4) compared to normal controls. b Waterfall plot depicting the top ten upregulated and downregulated genes in patients with FALS. c Volcano plot illustrating the statistical significance against the fold change for gene expression in patients with FALS. FALS, familial amyotrophic lateral sclerosis; RNA-seq, RNA sequencing

Techniques: RNA Sequencing, Expressing, Variant Assay, Gene Expression

DNAJC7 is required for the disassembly of stress-induced TDP-43 condensates. a Representative living-cell images showing TDP-43 ΔNLS-Clover condensates induced by arsenite treatment for 1 h, followed by the removal of stress in U2OS cells treated with control siRNA, or siRNAs targeting HSPA1A, HSPB1, BAG2, and DNAJC7 . b Percentage of cells forming TDP-43 condensates after the 1-h arsenite treatment. Data represents means from four independent experiments with individual values shown. c Percentage of cells in which TDP-43 condensates disassemble after 12 h of stress removal. Data represents means from four independent experiments with individual values shown. ** p < 0.01 (non-repeated measures analysis of variance and Bonferroni post hoc test). d Representative live-cell images showing TDP-43 ΔNLS-Clover condensates induced by arsenite treatment for 1 h, followed by stress removal in U2OS cells transduced with DNAJC7 -mCherry lentivirus to increase DNAJC7 levels. e Percentage of cells forming TDP-43 condensates after 1-h arsenite treatment. Data are shown as mean ± SD from two independent experiments. * p < 0.05 (two-tailed unpaired Student’s t -test). f Percentage of cells recovering and disassembling TDP-43 condensates after 12 h of stress removal. Data are shown as mean ± SD from two independent experiments. * p < 0.05 (two-tailed unpaired Student’s t -test)

Journal: Acta Neuropathologica

Article Title: Biallelic variants in DNAJC7 cause familial amyotrophic lateral sclerosis with the TDP-43 pathology

doi: 10.1007/s00401-025-02899-y

Figure Lengend Snippet: DNAJC7 is required for the disassembly of stress-induced TDP-43 condensates. a Representative living-cell images showing TDP-43 ΔNLS-Clover condensates induced by arsenite treatment for 1 h, followed by the removal of stress in U2OS cells treated with control siRNA, or siRNAs targeting HSPA1A, HSPB1, BAG2, and DNAJC7 . b Percentage of cells forming TDP-43 condensates after the 1-h arsenite treatment. Data represents means from four independent experiments with individual values shown. c Percentage of cells in which TDP-43 condensates disassemble after 12 h of stress removal. Data represents means from four independent experiments with individual values shown. ** p < 0.01 (non-repeated measures analysis of variance and Bonferroni post hoc test). d Representative live-cell images showing TDP-43 ΔNLS-Clover condensates induced by arsenite treatment for 1 h, followed by stress removal in U2OS cells transduced with DNAJC7 -mCherry lentivirus to increase DNAJC7 levels. e Percentage of cells forming TDP-43 condensates after 1-h arsenite treatment. Data are shown as mean ± SD from two independent experiments. * p < 0.05 (two-tailed unpaired Student’s t -test). f Percentage of cells recovering and disassembling TDP-43 condensates after 12 h of stress removal. Data are shown as mean ± SD from two independent experiments. * p < 0.05 (two-tailed unpaired Student’s t -test)

Techniques: Control, Transduction, Two Tailed Test

Journal: The Journal of Biological Chemistry

Article Title: The Hsp40 cochaperone DNAJC7 regulates polyglutamine aggregation and exhibits context-dependent effects on polyglycine aggregation

doi: 10.1016/j.jbc.2026.111292

Figure Lengend Snippet: A CRISPRi screen of molecular chaperones identifies DNAJC7 as a modifier of polyQ aggregation. A , workflow of CRISPRi screen to identify molecular chaperone modifiers of polyQ aggregation in NLS-FRET-Q79 cells. Created with BioRender. B , volcano plot CRISPRi screen results, highlighting DNAJ protein and proteasomal subunit hits. C , flow cytometry plots of NLS-FRET-Q79 cells at 4 days of doxycycline with or without proteasomal inhibitor (carfilzomib). D and E , Western blot ( D ) and quantification ( E ) of DNAJC7 levels in NLS-FRET-Q79 cells expressing nontargeting control (NTC) sgRNAs or sgRNAs targeting DNAJC7. Each lane represents lysate from an independent well; bars and error bars indicate mean ± SD, and points represent n = 3 wells. Signal intensities were normalized to total protein and then to sgNTC#1. F , results of flow cytometry measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with sgNTC or sgDNAJC7. Data are presented as fold change relative to the mean of the sgNTCs for each independent experiment. Bars and error bars represent mean ± SD, and points represent n = 3 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). CRISPRi, CRISPR interference. NLS, nuclear localization signal; polyQ, polyglutamine; sgRNA, single guide RNA.

Article Snippet: Primary antibodies include rabbit anti-DNAJC7 (Proteintech, 11090-1-AP, 1:2000 dilution) and mouse anti-GAPDH (Proteintech, 60004-1-Ig; 1:2000 dilution).

Techniques: Flow Cytometry, Western Blot, Expressing, Control, Transduction, CRISPR

Journal: The Journal of Biological Chemistry

Article Title: The Hsp40 cochaperone DNAJC7 regulates polyglutamine aggregation and exhibits context-dependent effects on polyglycine aggregation

doi: 10.1016/j.jbc.2026.111292

Figure Lengend Snippet: DNAJC7 suppresses mutant Huntingtin exon 1 (HTTex1) aggregation and colocalizes with inclusions. A , schematic of a lentiviral construct encoding a doxycycline-inducible eGFP-tagged fragment of HTTex1 containing 72 glutamines (GFP-HTTex1-Q72) used to generate a monoclonal HEK293T CRISPRi cell line. B , fluorescence micrograph of GFP-HTTex1-Q72 cells at 7 days of doxycycline treatment before and after treatment with Triton X-100. C , results of flow cytometry experiments measuring the fraction of total events containing detergent-resistant GFP + cells comparing those stably transduced with NTC or DNAJC7-targeting sgRNAs and normalized to the mean of NTCs. Data represent mean ± SD from n = 4 independent experiments. ∗∗ p < 0.05, one-sample t test compared with sgNTCs (fold change = 1.0). D , fluorescence micrographs of HEK293T cells 48 h after transient cotransfection of GFP-HTTex1-Q72 and either mTagBFP2 (BFP) alone or BFP-tagged DNAJC7. Arrows indicate cytoplasmic aggregates. E , flow cytometry plotting GFP-height versus GFP-width (pulse shape analysis) of HEK293T cells transfected with GFP-HTTex1-Q72 for 48 h. The detergent-resistant population of cells is designated as aggregate positive (Agg + ). F , results of flow cytometry experiments measuring Agg + cells in HEK293T cells 48 h after transient cotransfection with GFP-HTTex1-Q72 and either BFP or BFP-DNAJC7. Bars and error bars represent mean ± SD from n = 4 independent biological replicates (indicated by color), with each performed in technical triplicate. ∗ p < 0.05 by Student’s t test using mean values of technical replicates. The scale bars represent 10 μm. CRISPRi, CRISPR interference; eGFP, enhanced GFP; HEK293T, human embryonic kidney 293T cell line; NTC, nontargeting control; sgRNA, single guide RNA.

Article Snippet: Primary antibodies include rabbit anti-DNAJC7 (Proteintech, 11090-1-AP, 1:2000 dilution) and mouse anti-GAPDH (Proteintech, 60004-1-Ig; 1:2000 dilution).

Techniques: Mutagenesis, Construct, Fluorescence, Flow Cytometry, Stable Transfection, Transduction, Cotransfection, Transfection, CRISPR, Control

Journal: The Journal of Biological Chemistry

Article Title: The Hsp40 cochaperone DNAJC7 regulates polyglutamine aggregation and exhibits context-dependent effects on polyglycine aggregation

doi: 10.1016/j.jbc.2026.111292

Figure Lengend Snippet: Chaperone CRISPRi screening reveals limited suppression of polyG aggregation despite effects by DNAJC7 overexpression. A , volcano plot showing results of a CRISPRi screen for molecular chaperone modifiers of polyG aggregation in the NLS-FRET-G100 cell line. B , pairwise comparison of gene scores between the polyG screen (from A ) and the polyQ screen ( B ). C , results of flow cytometry experiments measuring fold change in the fraction of FRET-high cells after 5 days of doxycycline in sgRNA + cells transduced with NTC or DNAJC7. Data represent mean ± SD from n = 3 independent experiments. D , representative fluorescence micrographs of HEK293T cells transiently cotransfected with NLS-mScarlet- and NLS-mNeonGreen-tagged uN2C-G100 together with either BFP or BFP-DNAJC7; only the mNeonGreen channel is shown. The scale bar represents 10 μm. E , flow cytometry quantification of the percentage of FRET-high cells relative to total cells expressing both mNeonGreen and mScarlet. Bars indicate mean ± SD from n = 3 independent experiments. ∗ p < 0.05 by Student’s t test. CRISPRi, CRISPR interference; HEK293T, human embryonic kidney 293T cell line; NLS, nuclear localization signal; NTC, nontargeting control; polyG, polyglucine; polyQ, polyglutamine; sgRNA, single guide RNA.

Article Snippet: Primary antibodies include rabbit anti-DNAJC7 (Proteintech, 11090-1-AP, 1:2000 dilution) and mouse anti-GAPDH (Proteintech, 60004-1-Ig; 1:2000 dilution).

Techniques: Over Expression, Comparison, Flow Cytometry, Transduction, Fluorescence, Expressing, CRISPR, Control

Representative confocal micrographs of AD293 cells treated with 0.5 M sorbitol for 1 h. Cells transiently transfected with WDR62-mCherry and either the HSP co-chaperones ( A ) BAG2-EGFP, ( B ) EGFP-DNAJC7, ( C ) EGFP-STIP1, or de novo purine biosynthesis (DNPB) enzymes ( D ) PFAS-EGFP, ( E ) PPAT-EGFP, ( F ) GART-EGFP or ( G ) PAICS-EGFP. Co-localisation of each signal is indicated by fluorescence intensity plots to the right of each set of images ( y -axis represents fluorescence intensity (a.u.), x -axis (µm) represents the length of the white line drawn on the ROI). ( H ) Bar graph representing the co-localisation between WDR62-mCherry and EGFP signal for each respective protein (mean ± SD). Each dot represents the Person’s correlation coefficient for a single ROI. ( I ) Confocal micrographs of AD293 cells co-transfected with WDR62-mCherry and PFAS-EGFP, in either control (top) or purine-depleted (bottom) conditions. Bar graph on the right represents co-localisation between WDR62-mCherry and PFAS-EGFP (mean ± SD) ( ***P = 0.0004). ( J ) Co-immunoprecipitation and immunoblot of myc-PFAS and HA-WDR62. ( K ) The association of endogenous WDR62 with BAG2 and PFAS as measured by PLA (grey spots) with DAPI-stained nuclei in blue. PLA signal is observed under basal (control) and sorbitol-treated (0.5 M, 1 h) conditions. ( L ) Quantification of the number of PLA puncta per cell for WDR62/BAG2 and WDR62/PFAS. Data represent n = 3 biological replicates. P values calculated based on mean values using a one-way ANOVA for ( H ) and a two-tailed unpaired T test for ( I , L ) (* P < 0.05, ** P < 0.005, *** P < 0.001, **** P < 0.0001, n.s. is P > 0.05). All scale bars represent 20 µm. .

Journal: The EMBO Journal

Article Title: Microcephaly-associated protein WDR62 supports purine metabolism by interacting with co-chaperone BAG2

doi: 10.1038/s44318-026-00724-0

Figure Lengend Snippet: Representative confocal micrographs of AD293 cells treated with 0.5 M sorbitol for 1 h. Cells transiently transfected with WDR62-mCherry and either the HSP co-chaperones ( A ) BAG2-EGFP, ( B ) EGFP-DNAJC7, ( C ) EGFP-STIP1, or de novo purine biosynthesis (DNPB) enzymes ( D ) PFAS-EGFP, ( E ) PPAT-EGFP, ( F ) GART-EGFP or ( G ) PAICS-EGFP. Co-localisation of each signal is indicated by fluorescence intensity plots to the right of each set of images ( y -axis represents fluorescence intensity (a.u.), x -axis (µm) represents the length of the white line drawn on the ROI). ( H ) Bar graph representing the co-localisation between WDR62-mCherry and EGFP signal for each respective protein (mean ± SD). Each dot represents the Person’s correlation coefficient for a single ROI. ( I ) Confocal micrographs of AD293 cells co-transfected with WDR62-mCherry and PFAS-EGFP, in either control (top) or purine-depleted (bottom) conditions. Bar graph on the right represents co-localisation between WDR62-mCherry and PFAS-EGFP (mean ± SD) ( ***P = 0.0004). ( J ) Co-immunoprecipitation and immunoblot of myc-PFAS and HA-WDR62. ( K ) The association of endogenous WDR62 with BAG2 and PFAS as measured by PLA (grey spots) with DAPI-stained nuclei in blue. PLA signal is observed under basal (control) and sorbitol-treated (0.5 M, 1 h) conditions. ( L ) Quantification of the number of PLA puncta per cell for WDR62/BAG2 and WDR62/PFAS. Data represent n = 3 biological replicates. P values calculated based on mean values using a one-way ANOVA for ( H ) and a two-tailed unpaired T test for ( I , L ) (* P < 0.05, ** P < 0.005, *** P < 0.001, **** P < 0.0001, n.s. is P > 0.05). All scale bars represent 20 µm. .

Article Snippet: DNAJC7 (rabbit polyclonal) , Proteintech , 11090-1-AP.

Techniques: Transfection, Fluorescence, Control, Immunoprecipitation, Western Blot, Staining, Two Tailed Test

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