title: Sequential expression of genes involved in muscular dystrophies during human development.
abstract: To elucidate the normal and pathophysiological roles of genes involved in the aetiology of muscular dystrophies, we studied the expression of dystrophin, four sarcoglycans, beta-dystroglycan and merosin during early human development. These proteins are expressed mainly in skeletal muscles while dystrophin, beta-dystroglycan, delta-sarcoglycan and merosin are in cardiac and smooth muscles. Dystrophin, beta-, delta-sarcoglycan and beta-dystroglycan are first expressed in the myotome at the 4th week of human embryogenesis, followed by gamma-sarcoglycan and merosin at the 6th week of development; alpha-sarcoglycan appears only at the level of the muscular fibre at the end of the embryonic period.
authors.affiliation: URA CNRS 1923, Généthon, 1 rue de l'Internationale, BP 60, 91002, Evry, France.
authors.forename: M
authors.initials: M
authors.lastname: Durand
authors.forename: L
authors.initials: L
authors.lastname: Suel
authors.forename: J P
authors.initials: JP
authors.lastname: Barbet
authors.forename: J S
authors.initials: JS
authors.lastname: Beckmann
authors.forename: F
authors.initials: F
authors.lastname: Fougerousse
mesh_terms: D003598:Cytoskeletal Proteins / Q000096:biosynthesis / Q000235:genetics; D049030:Dystroglycans; D016189:Dystrophin / Q000096:biosynthesis / Q000235:genetics; D005314:Embryonic and Fetal Development; D018507:Gene Expression Regulation, Developmental; D005865:Gestational Age; D006321:Heart / Q000196:embryology; D006801:Humans; D007797:Laminin / Q000096:biosynthesis / Q000235:genetics; D008562:Membrane Glycoproteins / Q000096:biosynthesis / Q000235:genetics; D009124:Muscle Proteins / Q000096:biosynthesis* / Q000235:genetics; D018482:Muscle, Skeletal / Q000196:embryology / Q000378:metabolism; D009136:Muscular Dystrophies / Q000235:genetics*; D009206:Myocardium / Q000378:metabolism; D017434:Protein Structure, Tertiary; D049031:Sarcoglycans
chemical_list: D003598:Cytoskeletal Proteins; C483081:DAG1 protein, human; D016189:Dystrophin; D007797:Laminin; D008562:Membrane Glycoproteins; D009124:Muscle Proteins; D049031:Sarcoglycans; D049030:Dystroglycans