title: Selective defect in dystrophin-associated glycoproteins 50DAG (A2) and 35DAG (A4) in the dystrophic hamster: an animal model for severe childhood autosomal recessive muscular dystrophy (SCARMD).
abstract: To determine if dystrophin and dystrophin-associated glycoproteins (DAGs) are involved in muscle fiber necrosis in the dystrophic hamster, we examined NSJ-my/my (homozygous dystrophic) hamsters introduced from the BIO14.6 strain, by immunohistochemical and immunoblotting methods. Antibodies against dystrophin, utrophin and DAGs including 50DAG (A2), 43DAG (A3a) and 35DAG (A4) were employed for the examination. Dystrophin was stained strongly and utrophin stained very faintly along the sarcolemma of the dystrophic hamster, similar to the control. On the other hand, in the dystrophic hamster 50DAG (A2) and 35DAG (A4) were selectively defective, and 43DAG (A3a) was also decreased, although to a lesser degree. Since these results were almost identical to those seen in severe childhood autosomal recessive muscular dystrophy (SCARMD), the dystrophic hamster appears to be an animal model of SCARMD in which defects in DAGs may result in muscle fiber necrosis despite normal dystrophin expression.
authors.affiliation: Department of Ultrastructural Research, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Yamanouchi
authors.forename: Y
authors.initials: Y
authors.lastname: Mizuno
authors.forename: H
authors.initials: H
authors.lastname: Yamamoto
authors.forename: M
authors.initials: M
authors.lastname: Takemitsu
authors.forename: M
authors.initials: M
authors.lastname: Yoshida
authors.forename: I
authors.initials: I
authors.lastname: Nonaka
authors.forename: E
authors.initials: E
authors.lastname: Ozawa
mesh_terms: D000818:Animals; D000918:Antibody Specificity; D006224:Cricetinae; D003598:Cytoskeletal Proteins / Q000235:genetics*; D004195:Disease Models, Animal; D016189:Dystrophin / Q000235:genetics*; D004591:Electrophoresis, Polyacrylamide Gel; D015151:Immunoblotting; D007150:Immunohistochemistry; D008562:Membrane Glycoproteins / Q000235:genetics*; D008565:Membrane Proteins; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D049031:Sarcoglycans; D012508:Sarcolemma / Q000378:metabolism; D049411:Utrophin
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D049031:Sarcoglycans; D049411:Utrophin