ArticleKidney international reports2026
Alport Syndrome is a Partial Tubulointerstitial Disease of the Kidney.
Article in Kidney international reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- Kidney Transcriptome Sequencing Improves Molecular Diagnosis and Reveals Splicing Complexity Across the Alport Spectrum.Kidney international reports · 2026Article
- Unanswered Questions About Microscopic Hematuria With Tubulopathy.Kidney international reports · 2026Article
- Alporting Trouble Down the Tubules.Kidney international reports · 2026Article
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Authors and funding
15 authors.
Funding
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Abstract
Introduction: Recent genetic studies have shown that Alport syndrome (AS) is much more prevalent than clinically recognized, suggesting that atypical cases may phenocopy other kidney diseases. To date, pathomechanistic studies of AS have focused exclusively on the glomerular membrane, yet equally strong expression of collagen α(IV) chains is found along the distal renal tubule. We hypothesized that genetically determined abnormality of the tubular collagen IV (α345) molecule contributes to kidney failure and may drive atypical phenotypes. Methods: Histology and primary tubular cells (PTCs) of 8 patients with AS were investigated alongside controls. Results: Collagen α5 (IV) was detected within the tubular basement membrane (BM) (TBM) of the distal segments of renal tubules by immunohistochemistry. Conclusion: Our study demonstrates that the collagen IV (α345) molecule within the TBM is largely produced by the distal tubule itself. In AS, the TBM shows ultrastructural changes, which may induce fibrotic molecular signatures, as tubulointerstitial fibrosis appears to start in the vicinity of the distal tubule. Therefore, we postulate that the progression of kidney disease in AS may in part stem from the (distal) tubular apparatus.
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