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Loss of SMYD1 Results in Perinatal Lethality via Selective Defects within Myotonic Muscle Descendants.


ABSTRACT: SET and MYND Domain 1 (SMYD1) is a cardiac and skeletal muscle-specific, histone methyl transferase that is critical for both embryonic and adult heart development and function in both mice and men. We report here that skeletal muscle-specific, myogenin (myoG)-Cre-mediated conditional knockout (CKO) of Smyd1 results in perinatal death. As early as embryonic day 12.5, Smyd1 CKOs exhibit multiple skeletal muscle defects in proliferation, morphology, and gene expression. However, all myotonic descendants are not afflicted equally. Trunk muscles are virtually ablated with excessive accumulation of brown adipose tissue (BAT), forelimb muscles are disorganized and improperly differentiated, but other muscles, such as the masseter, are normal. While expression of major myogenic regulators went un

SUBMITTER: Rasmussen TL 

PROVIDER: S-EPMC6473627 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature