Alport Syndrome is a Partial Tubulointerstitial Disease of the Kidney
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.
Authors
- Michael Sean Wiesener (ORCID: https://orcid.org/0000-0003-4992-3132)
- Katharina A.E. Broeker (ORCID: https://orcid.org/0000-0001-5937-6756)
- Francesca Pasutto (ORCID: https://orcid.org/0000-0002-0026-5362)
- Daniel Reisenbüchler (ORCID: https://orcid.org/0000-0001-8820-0530)
- Florian J. Wopperer (ORCID: https://orcid.org/0000-0003-0065-6109)
- Stephanie Naas (ORCID: https://orcid.org/0000-0001-9301-6631)
- Dorit Merhof (ORCID: https://orcid.org/0000-0002-1672-2185)
- Karl X. Knaup (ORCID: https://orcid.org/0000-0003-1803-4463)
- Antje Wiesener (ORCID: https://orcid.org/0000-0003-1476-2260)
- Maike Buettner-Herold
- Mario Schiffer (ORCID: https://orcid.org/0000-0002-8414-1470)
- Nadja Kaiser
- Christoph Daniel (ORCID: https://orcid.org/0000-0002-6803-4755)
- Lisa Loderbauer
- Karen Schneider
Institutions
- TUM Klinikum (DE)
- Universitätsklinikum Erlangen (DE)
- Else Kröner-Fresenius-Stiftung (DE)
- University of Regensburg (DE)
Publication Details
- Journal
- Kidney International Reports
- Published
- 2025-11-20
- DOI
- https://doi.org/10.1016/j.ekir.2025.11.019
- Primary Topic
- Cell Adhesion Molecules Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00