A homologue of dystrophin is expressed at the neuromuscular junctions of normal individuals and DMD patients, and of normal and mdx mice Immunological evidence
Open Access
- 22 April 1991
- journal article
- case report
- Published by Wiley in FEBS Letters
- Vol. 282 (1), 161-165
- https://doi.org/10.1016/0014-5793(91)80468-i
Abstract
Polyclonal and monoclonal antibodies, which recognize different regions and epitopes of the dystrophin molecule, bind to a protein of M r 400 000 which is present in extracts of mdx muscle from regions which contain neuromuscular junctions (NMJ) and is absent from those which do not. This NMJ‐associated homologue of dystrophin has at least 2 epitopes which are different to the usual Xp21 form of dystrophin expressed along the sarcolemma of muscle fibres in normal muscles. This protein is also expressed at the NMJ of a DMD patient who lacks the first 52 exons of the Xp21 dystrophin gene and it must therefore be translated from a different gene transcript.Keywords
This publication has 29 references indexed in Scilit:
- Analysis of dystrophin in fast‐ and slow‐twitch skeletal muscles from mdx and dy2J mice at different agesMuscle & Nerve, 1990
- An autosomal transcript in skeletal muscle with homology to dystrophinNature, 1989
- Alternative splicing of human dystrophin mRNA generates isoforms at the carboxy terminusNature, 1989
- Detection of a specific isoform of alpha-actinin with antisera directed against dystrophin.The Journal of cell biology, 1989
- Immunohistochemical dystrophin reaction in synaptic regionsBrain & Development, 1989
- Duchenne muscular dystrophy: Deficiency of dystrophin at the muscle cell surfaceCell, 1988
- Characterization of Dystrophin in Muscle-Biopsy Specimens from Patients with Duchenne's or Becker's Muscular DystrophyNew England Journal of Medicine, 1988
- Negative immunostaining of Duchenne muscular dystrophy(DMD) and mdx muscle surface membrane with antibody against synthetic peptide fragment predicted from DMD cDNA.Proceedings of the Japan Academy, Series B, 1988
- Isolation of candidate cDNAs for portions of the Duchenne muscular dystrophy geneNature, 1986
- THE LOCALIZATION OF CHOLINESTERASE ACTIVITY IN RAT CARDIAC MUSCLE BY ELECTRON MICROSCOPYThe Journal of cell biology, 1964