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

Autor: Humphrey GW; Program in Physical Biology, Eunice Kennedy Schriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA., Mekhedov E, Blank PS, de Morree A, Pekkurnaz G, Nagaraju K, Zimmerberg J
Jazyk: angličtina
Zdroj: Experimental cell research [Exp Cell Res] 2012 Jan 15; Vol. 318 (2), pp. 127-35. Date of Electronic Publication: 2011 Oct 12.
DOI: 10.1016/j.yexcr.2011.10.004
Abstrakt: 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 (~200kDa) membrane-anchored protein, required for maintenance of plasmalemmal integrity in muscle fibers. To facilitate analysis of dysferlin function in muscle cells, we have established a dysferlin-deficient myogenic cell line (GREG cells) from the A/J mouse, a genetic model for dysferlinopathy. GREG cells have no detectable dysferlin expression, but proliferate normally in growth medium and fuse into functional myotubes in differentiation medium. GREG myotubes exhibit deficiencies in plasma membrane repair, as measured by laser wounding in the presence of FM1-43 dye. Under the wounding conditions used, the majority (~66%) of GREG myotubes lack membrane repair capacity, while no membrane repair deficiency was observed in dysferlin-normal C2C12 myotubes, assayed under the same conditions. We discuss the possibility that the observed heterogeneity in membrane resealing represents genetic compensation for dysferlin deficiency.
(Published by Elsevier Inc.)
Databáze: MEDLINE
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