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:


These biomarkers could potentially indicate early kidney damage and enable earlier initiation of kidney-protective treatments in children with Alport syndrome.

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