Serological Extracellular Matrix Fragments May Serve as Early Kidney Damage Biomarkers
June 4, 2025
Serological extracellular matrix fragments may serve as early kidney damage biomarkers in children with defects in Alport genes
Introduction
Alport syndrome is a genetic disorder caused by variants in COL4A3, COL4A4, or COL4A5, which encode type IV collagen. The α3.α4.α5(IV) collagen network is a critical structural component of the glomerular and tubular basement membranes within the kidney’s extracellular matrix (ECM). Variants in this network compromise basement membrane integrity, leading to cell injury, inflammation, and fibrotic remodeling of the surrounding interstitial matrix, which contributes to progressive kidney dysfunction.
We aim to identify serological protease-generated fragments of the ECM as potential early biomarkers of kidney damage.
Poster
Conclusion
The following biomarkers were altered in children with Alport syndrome, compared to healthy donors, before the onset of detectable proteinuria:
- C4M, LG1M, NIC (basement membrane degradation)
- nordicPRO-C3™ and nordicPRO-C6™ (interstitial matrix formation)
- C1SIG and C6M (interstitial matrix degradation)
- PRO-C4 (basement membrane turnover)
These biomarkers could potentially indicate early kidney damage and enable earlier initiation of kidney-protective treatments in children with Alport syndrome.