Decreased Proliferation Kinetics of Mouse Myoblasts Overexpressing FRG1

Chen, Steven C. and Frett, Ellie and Marx, Joseph and Bosnakovski, Darko and Reed, Xylena and Kyba, Michael and Kennedy, Brian K. and Feany, Mel B. (2011) Decreased Proliferation Kinetics of Mouse Myoblasts Overexpressing FRG1. Plos One.

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Although recent publications have linked the molecular events driving facioscapulohumeral muscular dystrophy (FSHD) to expression of the double homeobox transcription factor DUX4, overexpression of FRG1 has been proposed as one alternative causal agent as mice overexpressing FRG1 present with muscular dystrophy. Here, we characterize proliferative defects in two independent myoblast lines overexpressing FRG1. Myoblasts isolated from thigh muscle of FRG1 transgenic mice, an affected dystrophic muscle, exhibit delayed proliferation as measured by decreased clone size, whereas myoblasts isolated from the unaffected diaphragm muscle proliferated normally. To confirm the observation that overexpression of FRG1 could impair myoblast proliferation, we examined C2C12 myoblasts with inducible overexpression of FRG1, finding increased doubling time and G1-phase cells in mass culture after induction of FRG1 and decreased levels of pRb phosphorylation. We propose that depressed myoblast proliferation may contribute to the pathology of mice overexpressing FRG1 and may play a part in FSHD.

Item Type: Article
Subjects: Natural sciences > Biological sciences
Divisions: Faculty of Medical Science
Depositing User: Darko Bosnakovski
Date Deposited: 30 Nov 2012 13:47
Last Modified: 08 Feb 2013 09:02

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