Dystrophin-related protein in skeletal muscles in neuromuscular disorders: immunohistochemical study.
Thirty-four biopsied muscles of Duchenne, Becker and congenital muscular dystrophy, congenital myotonic dystrophy and amyotrophic lateral sclerosis were examined by an immunocytochemical method with an anti-dystrophin-related protein (DRP) antibody. Strongly positive immunoreaction to DRP at the neuromuscular junctions in all biopsied specimens and faint reaction on the surface membrane of atrophic fibers in amyotrophic lateral sclerosis suggest that DRP is an anchor protein of the acetylcholine receptor. Additionally, the surface membrane of muscle fibers of Duchenne muscular dystrophy was positively stained. DRP is, therefore, thought to be expressed to compensate for dystrophin deficiency in these muscle fibers.
Takemitsu
M
M
National Institute of Neuroscience, NCNP, Tokyo, Japan.
Nonaka
I
I
Sugita
H
H
D000595:Amino Acid Sequence; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism*; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D008565:Membrane Proteins; D008969:Molecular Sequence Data; D009132:Muscles / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism*; D009223:Myotonic Dystrophy / Q000378:metabolism*; D049411:Utrophin
D003598:Cytoskeletal Proteins; D008565:Membrane Proteins; D049411:Utrophin