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ADAMTS5 Orchestrates Cell Lineage Specific Patterning and Extracellular Matrix Organization During Semilunar Valve Development.


ABSTRACT: Aortic valve (AV) disease affects about 5% of the aging population, with AV replacement as the only treatment option. Histopathology indicates that accumulation of extracellular matrix (ECM) proteoglycans correlates with dysfunctional AVs. Proteoglycan content is controlled by ECM proteolytic cleavage, with the cleaved and intact forms of the proteoglycan Versican (VCAN) occupying different cell lineage-specific regions throughout AV development. To test the hypothesis that VCAN cleavage is required for lineage specific cell behaviors and ECM stratification, the cardiac neural crest (CNC) lineage was traced in mice with global inactivation of the proteoglycan protease Adamts5. By mid-gestation, Adamts5-/- mice exhibited disorganized CNC patterning with excess VCAN

SUBMITTER: Dupuis LE 

PROVIDER: S-EPMC12470463 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature