Evidence map›Paper›PMID 41531438›Full record

ArticleKidney international reports2026

Alport Syndrome is a Partial Tubulointerstitial Disease of the Kidney.

Lisa Loderbauer, Karl X Knaup, Daniel Reisenbüchler, Nicolas Kaiser, Stephanie Naas, Karen Schneider, Florian J Wopperer, Antje Wiesener, Francesca Pasutto, Mario Schiffer and 5 more

Abstract read
In one paragraph

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.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

3 citing papers in PubMed.

  1. Article
  2. Article
  3. Alporting Trouble Down the Tubules.Kidney international reports · 2026
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors.

Lisa LoderbauerDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.
Karl X KnaupDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.
Daniel ReisenbüchlerDepartment of Image Analysis and Computer Vision, University of Regensburg, Regensburg, Germany.
Nicolas KaiserDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.
Stephanie NaasDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.
Karen SchneiderDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.
Florian J WoppererDepartment of Nephrology, Center for Rare and Genetic Kidney Diseases, TUM University Hospital Rechts der Isar, TUM School of Medicine and Health, Munich, Germany.
Antje WiesenerInstitute of Human Genetics, University Hospital Erlangen, Erlangen, Germany.
Francesca PasuttoInstitute of Human Genetics, University Hospital Erlangen, Erlangen, Germany.
Mario SchifferDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.
Christoph DanielDepartment of Nephropathology, University Hospital Erlangen, Erlangen, Germany.
Katharina A E BroekerInstitute of Physiology, University of Regensburg, Regensburg, Germany.
Dorit MerhofDepartment of Image Analysis and Computer Vision, University of Regensburg, Regensburg, Germany.
Maike Buettner-HeroldDepartment of Nephropathology, University Hospital Erlangen, Erlangen, Germany.
Michael S WiesenerDepartment of Nephrology and Hypertension, University Hospital Erlangen, Erlangen, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

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.

Indexed as

extracellular matrixhearing impairmenthereditary nephritismicrohematuriaproteinuriathin basement

Identifiers

PMID41531438
PMCPMC12794056

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.