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Rescue of skeletal muscle alpha-actin-null mice by cardiac (fetal) alpha-actin.

The Journal of cell biology (2009-05-27)
Kristen J Nowak, Gianina Ravenscroft, Connie Jackaman, Aleksandra Filipovska, Stefan M Davies, Esther M Lim, Sarah E Squire, Allyson C Potter, Elizabeth Baker, Sophie Clément, Caroline A Sewry, Victoria Fabian, Kelly Crawford, James L Lessard, Lisa M Griffiths, John M Papadimitriou, Yun Shen, Grant Morahan, Anthony J Bakker, Kay E Davies, Nigel G Laing

Skeletal muscle alpha-actin (ACTA1) is the major actin in postnatal skeletal muscle. Mutations of ACTA1 cause mostly fatal congenital myopathies. Cardiac alpha-actin (ACTC) is the major striated actin in adult heart and fetal skeletal muscle. It is unknown why ACTC and ACTA1 expression switch during development. We investigated whether ACTC can replace ACTA1 in postnatal skeletal muscle. Two ACTC transgenic mouse lines were crossed with Acta1 knockout mice (which all die by 9 d after birth). Offspring resulting from the cross with the high expressing line survive to old age, and their skeletal muscles show no gross pathological features. The mice are not impaired on grip strength, rotarod, or locomotor activity. These findings indicate that ACTC is sufficiently similar to ACTA1 to produce adequate function in postnatal skeletal muscle. This raises the prospect that ACTC reactivation might provide a therapy for ACTA1 diseases. In addition, the mouse model will allow analysis of the precise functional differences between ACTA1 and ACTC.

Product Number
Product Description

Anti-Mouse IgG (whole molecule)–Peroxidase antibody produced in rabbit, IgG fraction of antiserum, buffered aqueous solution
Anti-Rabbit IgG (whole molecule)–Peroxidase antibody produced in goat, affinity isolated antibody