Spatial proteomics reveals recombinant human laminin-111 restores adhesion signaling to laminin-α2-deficient muscle.

ABSTRACT: Laminin-α2-related congenital muscular dystrophy (LAMA2-CMD) is a severe neuromuscular disorder caused by mutations in the LAMA2 gene, leading to loss of heterotrimers laminin-211/221, key components of the skeletal muscle extracellular matrix. Their absence disrupts adhesion between the cytoskeleton and extracellular matrix, resulting in progressive muscle wasting. Laminin-211/221 interacts with adhesion complexes such as the dystrophin/utrophin glycoprotein complex and α7β1-integrin. However, the regulatory mechanisms of these laminin-binding complexes and the broader role of laminin's influence on the formation of the macromolecular network in skeletal muscle remain unclear. We previously demonstrated that delivering mouse laminin-111 to the dyW-/- mouse model of LAMA2-CMD prevented dis

SUBMITTER: Hermann HJ 

PROVIDER: S-EPMC12643501 | biostudies-literature | 2025 Nov

REPOSITORIES: biostudies-literature