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Hum Mol Genet. 2009 Jan 15;18(2):318-27. doi: 10.1093/hmg/ddn358. Epub 2008 Nov 4.

Myogenic Akt signaling upregulates the utrophin-glycoprotein complex and promotes sarcolemma stability in muscular dystrophy.

Author information

1
Department of Physiological Science, University of California, Los Angeles, CA 90095, USA.

Abstract

Duchenne muscular dystrophy is caused by dystrophin mutations that lead to structural instability of the sarcolemma membrane, myofiber degeneration/regeneration and progressive muscle wasting. Here we show that myogenic Akt signaling in mouse models of dystrophy promotes increased expression of utrophin, which replaces the function of dystrophin thereby preventing sarcolemma damage and muscle wasting. In contrast to previous suggestions that increased Akt in dystrophy was a secondary consequence of pathology, our findings demonstrate a pivotal role for this signaling pathway such that modulation of Akt can significantly affect disease outcome by amplification of existing, physiological compensatory mechanisms.

PMID:
18986978
PMCID:
PMC2638781
DOI:
10.1093/hmg/ddn358
[Indexed for MEDLINE]
Free PMC Article

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