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).
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.
Yamanouchi
Y
Y
Department of Ultrastructural Research, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan.
Mizuno
Y
Y
Yamamoto
H
H
Takemitsu
M
M
Yoshida
M
M
Nonaka
I
I
Ozawa
E
E
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
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D049031:Sarcoglycans; D049411:Utrophin