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Dystrophin and related proteins
- Source :
- Current Opinion in Genetics & Development. 3:484-490
- Publication Year :
- 1993
- Publisher :
- Elsevier BV, 1993.
-
Abstract
- During the past year significant progress has been made in understanding how dystrophin deficiency leads to muscle cell necrosis in Duchenne muscular dystrophy and Becker muscular dystrophy. Dystrophin interacts with a glycoprotein complex spanning the muscle sarcolemma, effectively linking the actin cytoskeleton to the extracellular matrix. The carboxyl terminus of dystrophin is required for glycoprotein binding. Interestingly, at least three mRNAs transcribed from the distal end of the DMD gene in tissues other than muscle have been shown to encode this domain. Deficiency of a second component of the dystrophin-associated glycoprotein complex has been shown to occur in another muscle-wasting disorder, severe childhood autosomal recessive muscular dystrophy. Sequence analysis of the entire cDNA for the autosomal dystrophin-related protein utrophin has shown that dystrophin and utrophin are closely related. Furthermore, both of these proteins have been shown to bind to the same or a similar glycoprotein complex in muscle.
- Subjects :
- musculoskeletal diseases
congenital, hereditary, and neonatal diseases and abnormalities
Utrophin
Glycoprotein binding
biology
Duchenne muscular dystrophy
Membrane Proteins
musculoskeletal system
medicine.disease
Molecular biology
Muscular Dystrophies
Dystrophin
Cytoskeletal Proteins
Dystrophin-associated protein complex
Glycoprotein complex
Genetics
medicine
biology.protein
Animals
Humans
Muscular dystrophy
ITGA7
Developmental Biology
Subjects
Details
- ISSN :
- 0959437X
- Volume :
- 3
- Database :
- OpenAIRE
- Journal :
- Current Opinion in Genetics & Development
- Accession number :
- edsair.doi.dedup.....cc398866124c0e4060e24743748ba8be