ArticleThe Journal of biological chemistry2010
Identification of the NC1 domain of {alpha}3 chain as critical for {alpha}3{alpha}4{alpha}5 type IV collagen network assembly.
Article in The Journal of biological chemistry, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 23 citations in OpenAlex.
- Podocyte specific exon skipping after disease onset improves kidney pathology and function in a mouse model of Alport syndrome.Scientific reports · 2025Article
- Genetic reprogramming with stem cells regenerates glomerular epithelial podocytes in Alport syndrome.Life science alliance · 2024Article
- Identification of unique α4 chain structure and conserved antiangiogenic activity of α3NC1 type IV collagen in zebrafish.Developmental dynamics : an official publication of the American Association of Anatomists · 2023Article
- A Comparative Presentation of Mouse Models That Recapitulate Most Features of Alport Syndrome.Genes · 2022Review
- Genetic Disorders of the Extracellular Matrix: From Cell and Gene Therapy to Future Applications in Regenerative Medicine.Annual review of genomics and human genetics · 2022Review
- Identification ofFrontiers in genetics · 2022Article
- The triple helix of collagens - an ancient protein structure that enabled animal multicellularity and tissue evolution.Journal of cell science · 2018Review
- Endothelin-1 mediated induction of extracellular matrix genes in strial marginal cells underlies strial pathology in Alport mice.Hearing research · 2016Article
- Endothelin A receptor activation on mesangial cells initiates Alport glomerular disease.Kidney international · 2016Article
- Type IV Collagen Controls the Axogenesis of Cerebellar Granule Cells by Regulating Basement Membrane Integrity in Zebrafish.PLoS genetics · 2015Article
- COL4A3/COL4A4 mutations and features in individuals with autosomal recessive Alport syndrome.Journal of the American Society of Nephrology : JASN · 2013Article
- Prognostic value of glomerular collagen IV immunofluorescence studies in male patients with X-linked Alport syndrome.Clinical journal of the American Society of Nephrology : CJASN · 2013Article
- Alport syndrome--insights from basic and clinical research.Nature reviews. Nephrology · 2013Review
- Goodpasture's disease: molecular architecture of the autoantigen provides clues to etiology and pathogenesis.Current opinion in nephrology and hypertension · 2011Review
Corrections and comments
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Authors and funding
11 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The network organization of type IV collagen consisting of α3, α4, and α5 chains in the glomerular basement membrane (GBM) is speculated to involve interactions of the triple helical and NC1 domain of individual α-chains, but in vivo evidence is lacking. To specifically address the contribution of the NC1 domain in the GBM collagen network organization, we generated a mouse with specific loss of α3NC1 domain while keeping the triple helical α3 chain intact by connecting it to the human α5NC1 domain. The absence of α3NC1 domain leads to the complete loss of the α4 chain. The α3 collagenous domain is incapable of incorporating the α5 chain, resulting in the impaired organization of the α3α4α5 chain-containing network. Although the α5 chain can assemble with the α1, α2, and α6 chains, such assembly is incapable of functionally replacing the α3α4α5 protomer. This novel approach to explore the assembly type IV collagen in vivo offers novel insights in the specific role of the NC1 domain in the assembly and function of GBM during health and disease.
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