Dystrophin, utrophin and beta-dystroglycan expression in skeletal muscle from patients with Becker muscular dystrophy.
J Neuropathol Exp Neurol
; 55(8): 896-903, 1996 Aug.
Article
em En
| MEDLINE
| ID: mdl-8759779
ABSTRACT
The precise localization and semiquantitative correlation of dystrophin, utrophin and beta-dystroglycan expression on the sarcolemma of skeletal muscle cells obtained from patients with Becker muscular dystrophy (BMD) was studied using three types of double immunofluorescence. Staining intensity was measured using a confocal laser microscope. Each of these proteins was identified at the same locus on the sarcolemma. The staining intensities of dystrophin and utrophin were approximately reciprocal at sarcolemmal sites where dystrophin expression was obviously observed. The staining intensity of beta-dystroglycan was strong in areas where dystrophin staining was also strong and utrophin expression was weak. Quantitative analysis revealed that the staining intensity of beta-dystroglycan minus that of dystrophin approximated the staining intensity of utrophin, indicating that the sum of dystrophin and utrophin expression corresponds to that of beta-dystroglycan. These results suggest that utrophin may compensate for dystrophin deficiency found in BMD by binding to beta-dystroglycan.
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Base de dados:
MEDLINE
Assunto principal:
Glicoproteínas de Membrana
/
Distrofina
/
Músculo Esquelético
/
Proteínas do Citoesqueleto
/
Proteínas de Membrana
/
Distrofias Musculares
Limite:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Humans
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Male
/
Middle aged
Idioma:
En
Ano de publicação:
1996
Tipo de documento:
Article