GREG cells, a dysferlin-deficient myogenic mouse cell line.

摘要: Abstract The dysferlinopathies (e.g. LGMD2b, Myoshi myopathy) are progressive, adult-onset muscle wasting syndromes caused by mutations in the gene coding for dysferlin. Dysferlin is a large (~ 200 kDa) membrane-anchored protein, required maintenance of plasmalemmal integrity fibers. To facilitate analysis dysferlin function cells, we have established dysferlin-deficient myogenic cell line (GREG cells) from A/J mouse, genetic model dysferlinopathy. GREG cells no detectable expression, but proliferate normally growth medium and fuse into functional myotubes differentiation medium. exhibit deficiencies plasma membrane repair, as measured laser wounding presence FM1–43 dye. Under conditions used, majority (~ 66%) lack repair capacity, while deficiency was observed dysferlin-normal C2C12 myotubes, assayed under same conditions. We discuss possibility that heterogeneity resealing represents compensation deficiency.