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Immunostaining of skeletal and cardiac muscle surface membrane with antibody against Duchenne muscular dystrophy peptide

Immunostaining of skeletal and cardiac muscle surface membrane with antibody against Duchenne... Duchenne muscular dystrophy (DMD) is a debilitating X-linked muscle disease. We have used sequence information from complementary DNA clones, derived from the gene that is deleted in DMD patients1–3, to generate an antiserum that stains the surface membrane of intact human and mouse skeletal muscle, but not that of DMD patients and mdx mice4. Here we identify the protein reacting with this antiserum as a single component of relative molecular mass 210,000 (M r = 210K) that fractionates with a low-ionic strength extract of intact human and mouse skeletal muscle. It is therefore distinct from the 400K protein found in the heavy microsomal fraction of normal muscle and identified as a putative product of the DMD gene5. We also analyse further the disease specificity of the antiserum. Positive staining is seen in normal controls, and in samples from patients with a wide range of muscular dystrophies other than DMD. Becker muscular dystrophy6, which is allelically related to DMD, was the only other exception, and gave a sporadic staining pattern. The demonstration of a specific defect in the surface membrane of DMD muscle fibres substantiates the hypothesis7–10 that membrane lesions may initiate muscle degradation in DMD. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Nature Springer Journals

Immunostaining of skeletal and cardiac muscle surface membrane with antibody against Duchenne muscular dystrophy peptide

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References (13)

Publisher
Springer Journals
Copyright
Copyright © 1988 by Nature Publishing Group
Subject
Science, Humanities and Social Sciences, multidisciplinary; Science, Humanities and Social Sciences, multidisciplinary; Science, multidisciplinary
ISSN
0028-0836
eISSN
1476-4687
DOI
10.1038/333861a0
Publisher site
See Article on Publisher Site

Abstract

Duchenne muscular dystrophy (DMD) is a debilitating X-linked muscle disease. We have used sequence information from complementary DNA clones, derived from the gene that is deleted in DMD patients1–3, to generate an antiserum that stains the surface membrane of intact human and mouse skeletal muscle, but not that of DMD patients and mdx mice4. Here we identify the protein reacting with this antiserum as a single component of relative molecular mass 210,000 (M r = 210K) that fractionates with a low-ionic strength extract of intact human and mouse skeletal muscle. It is therefore distinct from the 400K protein found in the heavy microsomal fraction of normal muscle and identified as a putative product of the DMD gene5. We also analyse further the disease specificity of the antiserum. Positive staining is seen in normal controls, and in samples from patients with a wide range of muscular dystrophies other than DMD. Becker muscular dystrophy6, which is allelically related to DMD, was the only other exception, and gave a sporadic staining pattern. The demonstration of a specific defect in the surface membrane of DMD muscle fibres substantiates the hypothesis7–10 that membrane lesions may initiate muscle degradation in DMD.

Journal

Nature – Springer Journals

Published: Jun 30, 1988

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