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Dev Dyn. June 1, 2009; 238 (6): 1502-12.

Muscular dystrophy candidate gene FRG1 is critical for muscle development.

Hanel ML , Wuebbles RD , Jones PL .

The leading candidate gene responsible for facioscapulohumeral muscular dystrophy (FSHD) is FRG1 (FSHD region gene 1). However, the correlation of altered FRG1 expression levels with disease pathology has remained controversial and the precise function of FRG1 is unknown. Here, we carried out a detailed analysis of the normal expression patterns and effects of FRG1 misexpression during vertebrate embryonic development using Xenopus laevis. We show that frg1 is expressed in and essential for the development of the tadpole musculature. FRG1 morpholino injection disrupted myotome organization and led to inhibited myotome growth, while elevated FRG1 led to abnormal epaxial and hypaxial muscle formation. Thus, maintenance of normal FRG1 levels is critical for proper muscle development, supportive of FSHD disease models whereby misregulation of FRG1 plays a causal role underlying the pathology exhibited in FSHD patients. Developmental Dynamics 238:1502-1512, 2009. (c) 2008 Wiley-Liss, Inc.

PubMed ID: 19097195
PMC ID: PMC2964887
Article link: Dev Dyn.
Grant support: 1R01AR055877 NIAMS NIH HHS , R01 AR055877-02 NIAMS NIH HHS

Genes referenced: fmo1 frg1 myf5 myod1 pax3 vim vim.2

Antibodies referenced: Frg1 Ab1 Somite Ab1 Vim Ab1
Morpholinos referenced: frg1 MO1 frg1 MO2

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