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Muscular dystrophy candidate gene FRG1 is critical for muscle development.
- Source :
-
Developmental dynamics : an official publication of the American Association of Anatomists [Dev Dyn] 2009 Jun; Vol. 238 (6), pp. 1502-12. - Publication Year :
- 2009
-
Abstract
- 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.
- Subjects :
- Animals
Humans
In Situ Hybridization
Microfilament Proteins
Muscle, Skeletal abnormalities
Muscle, Skeletal anatomy & histology
MyoD Protein genetics
MyoD Protein metabolism
Nuclear Proteins metabolism
Oligonucleotides, Antisense genetics
Oligonucleotides, Antisense metabolism
PAX3 Transcription Factor
Paired Box Transcription Factors genetics
Paired Box Transcription Factors metabolism
RNA-Binding Proteins
Xenopus Proteins genetics
Gene Expression Regulation, Developmental
Muscle Development genetics
Muscle, Skeletal embryology
Muscle, Skeletal growth & development
Muscular Dystrophy, Facioscapulohumeral genetics
Nuclear Proteins genetics
Xenopus Proteins metabolism
Xenopus laevis anatomy & histology
Xenopus laevis physiology
Subjects
Details
- Language :
- English
- ISSN :
- 1058-8388
- Volume :
- 238
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Developmental dynamics : an official publication of the American Association of Anatomists
- Publication Type :
- Academic Journal
- Accession number :
- 19097195
- Full Text :
- https://doi.org/10.1002/dvdy.21830