Immunocytochemical study of nebulin in Duchenne muscular dystrophy.
We used antibodies to nebulin in immunocytochemical studies. In normal muscle, nebulin was localized at the I band. The protein was also present in most fibers from all 15 Duchenne muscular dystrophy (DMD) patients studied, including patients who seemed to lack nebulin in electrophoretic gels and patients who demonstrated deletions of DNA in the region of Xp21. These results conform to other evidence that nebulin is not the primarily affected gene product in DMD; it may be affected secondarily.
Bonilla
E
E
H. Houston Merritt Clinical Research Center for Muscular Dystrophy and Related Diseases, New York, NY.
Miranda
A F
AF
Prelle
A
A
Salviati
G
G
Betto
R
R
Zeviani
M
M
Schon
E A
EA
DiMauro
S
S
Rowland
L P
LP
D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D006801:Humans; D007150:Immunohistochemistry; D009124:Muscle Proteins / Q000032:analysis*; D009136:Muscular Dystrophies / Q000378:metabolism*; D014960:X Chromosome
D009124:Muscle Proteins; C035620:nebulin