ArticleThe American journal of pathology2000
Glomerular expression of type IV collagen chains in normal and X-linked Alport syndrome kidneys.
Article in The American journal of pathology, 2000. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 93 citations in OpenAlex.
- The life cycle of type IV collagen.Matrix biology : journal of the International Society for Matrix Biology · 2025Review
- Genotype-Based Molecular Mechanisms in Alport Syndrome.Journal of the American Society of Nephrology : JASN · 2025Review
- Review
- Effects of a Novel COL4A3 Homozygous/Heterozygous Splicing Mutation on the Mild Phenotype in a Family With Autosomal Recessive Alport Syndrome and a Literature Review.Molecular genetics & genomic medicine · 2025Review
- Case Report: Neonatal nephropathy with polycystic appearance in child harboring WT1 variant.Frontiers in pediatrics · 2025Article
- Article
- Exploration of Gene Therapy for Alport Syndrome.Biomedicines · 2024Review
- A NovelGenes · 2024Article
- Peroxidasin-mediated bromine enrichment of basement membranes.Proceedings of the National Academy of Sciences of the United States of America · 2020Article
- Collagen IV Gene Mutations in Adults With Bilateral Renal Cysts and CKD.Kidney international reports · 2020Article
- Endothelial cell-specific collagen type IV-αAmerican journal of physiology. Renal physiology · 2019Article
- Features of Autosomal Recessive Alport Syndrome: A Systematic Review.Journal of clinical medicine · 2019Article
- Binding Truths: Atypical Anti-Glomerular Basement Membrane Disease Mediated by IgA Anti-Glomerular Basement Membrane Antibodies Targeting the α1 Chain of Type IV Collagen.Kidney international reports · 2019Article
- Basement membranes in the cornea and other organs that commonly develop fibrosis.Cell and tissue research · 2018Review
- Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study.BMC nephrology · 2018Article
- The Chemical Chaperone, PBA, Reduces ER Stress and Autophagy and Increases Collagen IV α5 Expression in Cultured Fibroblasts From Men With X-Linked Alport Syndrome and Missense Mutations.Kidney international reports · 2017Article
- Alport syndrome: facts and opinions.F1000Research · 2017Review
- Pathological features of proteinuric nephropathy resembling Alport syndrome in a young Pyrenean Mountain dog.The Journal of veterinary medical science · 2015Article
- A mouse Col4a4 mutation causing Alport glomerulosclerosis with abnormal collagen α3α4α5(IV) trimers.Kidney international · 2014Article
- Glomerular cell cross-talk influences composition and assembly of extracellular matrix.Journal of the American Society of Nephrology : JASN · 2014Article
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Alport syndrome is an inherited nephropathy characterized by alterations of the glomerular basement membrane because of mutations in type IV collagen genes. COL4A5 mutations, causing X-linked Alport syndrome, frequently result in the loss of the alpha5 chains of type IV collagen in basement membranes. This is associated with the absence of the alpha3(IV) and alpha4(IV) chains and increased amounts of alpha1(IV) and alpha2(IV) in glomerular basement membranes. The mechanisms resulting in such a configuration are still controversial and are of fundamental importance for understanding the pathology of the disease and for considering gene therapy. In this article we studied, for the first time, type IV collagen expression in kidneys from X-linked Alport syndrome patients, using in situ hybridization and immunohistochemistry. We show that, independent of the type of mutation and of the level of COL4A5 transcription, both COL4A3 and COL4A4 genes are actively transcribed in podocytes. Moreover, using immunofluorescence amplification, we were able to demonstrate that the alpha3 chain of type IV collagen was present in the podocytes of all patients. Finally, the alpha1(IV) chain, which accumulates within glomerular basement membranes, was found to be synthesized by mesangial/endothelial cells. These results strongly suggest that, contrary to what has been found in dogs affected with X-linked Alport syndrome, there is no transcriptional co-regulation of COL4A3, COL4A4, and COL4A5 genes in humans, and that the absence of alpha3(IV) to alpha5(IV) in glomerular basement membranes in the patients results from events downstream of transcription, RNA processing, and protein synthesis.
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