The Journal of cell biology · 2009
Rescue of skeletal muscle alpha-actin-null mice by cardiac (fetal) alpha-actin.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Nowak Kristen J, Ravenscroft Gianina, Jackaman Connie, Filipovska Aleksandra, Davies Stefan M, Lim Esther M, Squire Sarah E, Potter Allyson C, Baker Elizabeth, Clément Sophie, Sewry Caroline A, Fabian Victoria, Crawford Kelly, Lessard James L, Griffiths Lisa M, Papadimitriou John M, Shen Yun, Morahan Grant, Bakker Anthony J, Davies Kay E, Laing Nigel G
Аннотация
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.