UNIGE document Scientific Article
previous document  unige:5583  next document
add to browser collection

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

Nowak, Kristen J.
Ravenscroft, Gianina
Jackaman, Connie
Filipovska, Aleksandra
Davies, Stefan M.
Lim, Esther M.
Squire, Sarah E.
Potter, Allyson C.
show hidden authors show all authors [1 - 21]
Published in The Journal of Cell Biology. 2009, vol. 185, no. 5, p. 903-15
Abstract 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.
Keywords Actins/genetics/physiologyAnimalsGene Expression ProfilingHeart/embryologyMiceMice, KnockoutMuscle, Skeletal/metabolism/ultrastructureMyocardium/metabolism/ultrastructureProtein Isoforms/physiology
PMID: 19468071
Full text
(ISO format)
NOWAK, Kristen J. et al. Rescue of skeletal muscle alpha-actin-null mice by cardiac (fetal) alpha-actin. In: The Journal of Cell Biology, 2009, vol. 185, n° 5, p. 903-15. https://archive-ouverte.unige.ch/unige:5583

191 hits



Deposited on : 2010-03-25

Export document
Format :
Citation style :