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May 18, 2026Cell Reports1 citationsOpen Access

Accelerated basement membrane remodeling and serum matrix fragments as biomarkers of fibrosis in Alport syndrome

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RPRebecca PrestonAHAnna HoyleEWE. G. Williams

Key Points

  • This research aims to investigate the role of serum matrix fragments and basement membrane remodeling as biomarkers of fibrosis in Alport syndrome.
  • C-lysine proteomics assessed abundance and turnover of basement membrane components.
  • Super-resolution imaging evaluated matrix protein organization.
  • Peptide location fingerprinting analyzed damage modifications across approximately 40 matrix proteins.
  • Detected serum matrix fragments in children with Alport variants compared to healthy controls with statistical significance.
  • Identified fragmentation in collagens, laminins, and nidogens linked to accelerated basement membrane turnover.
  • Predicted biomarkers provide insights into chronic kidney disease and other fibrotic disorders.

Abstract

C-lysine proteomics demonstrated altered abundance and accelerated turnover of basement membrane components. Super-resolution imaging confirmed matrix protein disorganization, and peptide location fingerprinting mapped damage modifications across ∼40 matrix proteins, predicting fragmentation in collagens, laminins, and nidogens. Predicted matrix fragments were detectable in serum from children with Alport variants versus healthy controls, linking basement membrane turnover and fibrosis with clinically accessible biomarkers for CKD and other fibrotic disorders.

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Cite This Study

Preston et al. (2026) studied this question.

synapsesocial.com/papers/6a0aabc25ba8ef6d83b6f66dhttps://doi.org/10.1016/j.celrep.2026.117356
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