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Expression profiling of FRG1 transgenic mice.

ABSTRACT: RNA from skeletal muscle of mice over-expressing selectively in the skeletal muscle the gene FRG1, a candidate of facioscapulohumeral muscular dystrophy, was compared to RNA from skeletal muscle of WT mice. Total RNA obtained from isolated vastus lateralis muscles of 4 weeks old females WT mice and vastus lateralis muscles of 4 weeks old mice over-expressing FRG1, FSHD region gene 1.

ORGANISM(S): Mus musculus

SUBMITTER: Danilo Licastro

PROVIDER: E-GEOD-28575 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Overexpression of facioscapulohumeral muscular dystrophy region gene 1 causes primary defects in myogenic stem cells.

Xynos Alexandros A Neguembor Maria Victoria MV Caccia Roberta R Licastro Danilo D Nonis Alessandro A Di Serio Clelia C Stupka Elia E Gabellini Davide D

Journal of cell science 20130322 Pt 10

Overexpression of facioscapulohumeral muscular dystrophy region gene 1 (FRG1) in mice, frogs and worms leads to muscular and vascular abnormalities. Nevertheless, the mechanism that follows FRG1 overexpression and finally leads to muscular defects is currently unknown. Here, we show that the earliest phenotype displayed by mice overexpressing FRG1 is a postnatal muscle-growth defect. Long before the development of muscular dystrophy, FRG1 mice also exhibit a muscle regeneration impairment. Ex vi ...[more]

PMID: 23525014

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Rbfox1 downregulation and altered Calpain 3 splicing by FRG1 in a mouse model of facioscapulohumeral muscular dystrophy (FSHD).

Project description:FRG1 over-expression results in an FSHD-like phenotype in mice. Muscles (vastus lateralis and biceps brachii) from wild type and FRG1 over-expressing mice were compared at 4 and 13 weeks of age by splicing sensitive microarray.

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