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Dysregulated ATX-LPA and YAP/TAZ signaling in dystrophic Sgcd<sup>-/-</sup> mice with early fibrosis and inflammation.


ABSTRACT:

Background

Sarcoglycanopathies are muscle dystrophies caused by mutations in the genes encoding sarcoglycans (α, β, γ, and δ) that can destabilize the dystrophin-associated glycoprotein complex at the sarcolemma, leaving muscle fibers vulnerable to damage after contraction, followed by inflammatory and fibrotic responses and resulting in muscle weakness and atrophy. Two signaling pathways have been implicated in fibrosis and inflammation in various tissues: autotaxin/lysophosphatidic acid (ATX-LPA) and yes-associated protein 1/transcriptional co-activator with PDZ-binding motif (YAP/TAZ). LPA, synthesized by ATX, can act as a pleiotropic molecule due to its multiple receptors. Two Hippo pathway effectors, YAP/TAZ, can be dephosphorylated by LPA and translocated to the nucleus. They

SUBMITTER: Gutierrez-Rojas C 

PROVIDER: S-EPMC11884125 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature