Regulation of Skeletal Muscle Sarcomere Integrity and Postnatal Muscle Function by Mef2c

• DOI: 10.1128/mcb.01187-07
• Publication Date: 2007-09-18T01:19:17Z
• Authors: Matthew J. Potthoff; Michael A. Arnold; John McAnally; James A. Richardson; Rhonda Bassel-Duby; Eric N. Olson
• Keywords: MEF2C; Mef2; Myogenic regulatory factors; Dysferlin; GATA4

Myocyte enhancer factor 2 (MEF2) transcription factors cooperate with the MyoD family of basic helix-loop-helix (bHLH) to drive skeletal muscle development during embryogenesis, but little is known about potential functions MEF2 in postnatal muscle. Here we show that muscle-specific deletion Mef2c mice results disorganized myofibers and perinatal lethality. In contrast, neither Mef2a nor Mef2d required for normal vivo. Skeletal deficient differentiates forms rapidly deteriorate after birth due sarcomeres a loss integrity M line. Microarray analysis null muscles identified several structural genes depend on MEF2C, including those encoding M-line-specific proteins myomesin protein. We MEF2C directly regulates gene improper sarcomere organization. These reveal key role maintenance maturation