Kidney International Reports· 2026Q1
Proteinuria severity in Alport spectrum subtypes
- 0citations
- Q1SCImago
- 2026year
Short summary
Alport syndrome subtypes, particularly those linked to COL4A3/COL4A4 variants, exhibit varying proteinuria severity, prompting a proposed reclassification to 'Alport Risk-COL4A3 or COL4A4'.
AI-generated from the title and abstract; the full text is not read.
Key points
- Alport syndrome is caused by variants in COL4A5 (X-linked), COL4A3, and COL4A4 (autosomal).
- Proteinuria severity varies across different Alport syndrome subtypes.
- The term 'autosomal dominant AS' is considered inaccurate for describing the disease.
- A new classification, 'Alport Risk-COL4A3 or COL4A4', is proposed.
AI-generated from the title and abstract; the full text is not read.
Abstract
Alport syndrome (AS) is the most common inherited glomerular disease,1 caused by pathogenic (P) or likely pathogenic (LP) variants in three collagen IV genes: COL4A5, which leads to X-linked AS (XLAS), and COL4A3 and COL4A4, responsible for autosomal dominant AS (ADAS), and autosomal recessive AS (ARAS). Regarding the term ADAS, it is widely recognized that it does not accurately reflect the underlying disease.2 A recent publication based on international consensus has proposed the term Alport Risk-COL4A3 or COL4A4.
The authors' abstract, as published at the source. Kidney International Reports, 2026 · DOI ↗
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Field: Immunology and Allergy
Immunology and AllergyMedicine