Monoclonal antibodies recognizing dystrophin and dystrophin-associated protein A1.
نویسندگان
چکیده
منابع مشابه
The dystrophin-associated protein complex.
The lethal muscle-wasting disorder, Duchenne muscular dystrophy, is caused by mutations or deletions in the dystrophin gene. In skeletal and cardiac muscle, dystrophin associates with various proteins to form the dystrophinassociated protein complex (DAPC). The DAPC is thought to play a structural role in linking the actin cytoskeleton to the extracellular matrix, stabilizing the sarcolemma dur...
متن کاملDystrophin is a microtubule-associated protein
Cytolinkers are giant proteins that can stabilize cells by linking actin filaments, intermediate filaments, and microtubules (MTs) to transmembrane complexes. Dystrophin is functionally similar to cytolinkers, as it links the multiple components of the cellular cytoskeleton to the transmembrane dystroglycan complex. Although no direct link between dystrophin and MTs has been documented, costame...
متن کاملAssembly of the Dystrophin-Associated Protein Complex Does Not Require the Dystrophin Cooh-Terminal Domain
Dystrophin is a multidomain protein that links the actin cytoskeleton to laminin in the extracellular matrix through the dystrophin associated protein (DAP) complex. The COOH-terminal domain of dystrophin binds to two components of the DAP complex, syntrophin and dystrobrevin. To understand the role of syntrophin and dystrobrevin, we previously generated a series of transgenic mouse lines expre...
متن کاملThe concomitant use of dystrophin and utrophin/dystrophin related protein antibodies to reduce misdiagnosis of Duchenne/Becker muscular dystrophy.
Antibodies to dystrophin have increased accuracy in the diagnosis of Duchenne/Becker muscular dystrophy (D/BMD). Both typical and 'atypical' presentations of this disease can be confirmed by demonstrating qualitative and quantitative defects in the expression of dystrophin protein. However, owing to the propensity for dystrophin degradation in vitro, caution needs to be applied while performing...
متن کاملDystrophin analysis using a panel of anti-dystrophin antibodies in Duchenne and Becker muscular dystrophy.
Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, was studied in 19 patients with Xp21 disorders and in 25 individuals with non-Xp21 muscular dystrophy. Antibodies raised to seven different regions spanning most of the protein were used for immunocytochemistry. In all patients specific dystrophin staining anomalies were detected and correlated with clinical severity...
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ژورنال
عنوان ژورنال: Japanese Journal of Pharmacology
سال: 1991
ISSN: 0021-5198
DOI: 10.1016/s0021-5198(19)39357-6