Muscle basement membrane components and cell-surface receptors

The muscle basement membrane and its associated cell-surface receptors form a critical structural and functional unit that maintains the integrity of skeletal and cardiac muscle fibers (Sanes, J. R., 2003, Nature Reviews Neuroscience). This complex includes extracellular matrix (ECM) proteins such as laminin-211, collagen IV, and perlecan, which interact with transmembrane receptors like the dystroglycan complex and alpha7beta1 integrin (Yurchenco, P. D., 2011, Cold Spring Harbor Perspectives in Biology). These interactions facilitate force transmission from the cytoskeleton to the ECM and initiate signaling pathways essential for cell survival and regeneration. Defects in these components, such as mutations in the LAMA2 or COL6 genes, lead to various forms of muscular dystrophy characterized by membrane fragility and progressive muscle wasting (Mercuri, E., et al., 2019, Nature Reviews Disease Primers). While not a single drug target, this system is the focus of numerous therapeutic strategies, including gene replacement, protein substitution, and the use of small molecules to stabilize the membrane or reduce secondary fibrosis. This entry is considered 'incorrect' as a single target because it encompasses a broad category of multiple distinct proteins and receptors rather than a specific molecular entity.

Other names

Muscle basal laminaMyofiber extracellular matrixDystrophin-associated glycoprotein complex (DAGC) interactorsMuscle ECM-receptor unit

Modulation of structural integrity, replacement of missing structural proteins, or inhibition of pro-fibrotic signaling within the extracellular matrix.