Journal article
Absence of γ-sarcoglycan (35 DAG) in autosomal recessive muscular dystrophy linked to chromosome 13q12
FEBS letters, Vol.381(1), pp.15-20
1996
DOI: 10.1016/0014-5793(96)00056-7
PMID: 8641426
Abstract
We have partially sequenced rabbit skeletal muscle γ-sarcoglycan an integral component of the dystrophin-glycoprotein complex. Specific antibodies were produced against a γ-sarcoglycan peptide and used to examine the expression of γ-sarcoglycan in skeletal muscle of patients with severe childhood autosomal muscular dystrophy linked to chromosome 13q12 (SCARMD). We show by immunofluorescence and Western blotting that in skeletal muscle from these patients γ-sarcoglycan is completely absent and α- and β-sarcoglycan are greatly reduced in abundance, whereas other components of the DGC are preserved. In addition, we show that in normal muscle α-, β-, and γ-sarcoglycan constitute a tightly associated sarcolemma complex which can not be disrupted by SDS treatment.
Details
- Title: Subtitle
- Absence of γ-sarcoglycan (35 DAG) in autosomal recessive muscular dystrophy linked to chromosome 13q12
- Creators
- Daniel Jung - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USAFrance Leturcq - Laboratoire de Biochimie Génétique et INSERM 129, CHU Cochin, Université René Descartes, 75014 Paris, FranceYoshihide Sunada - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USAFranck Duclos - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USAFernando M.S Tomé - INSERM 153, 17 rue du Fer à Moulin, 75005 Paris, FranceCarolyn Moomaw - Howard Hughes Medical Institute, Biopolymer Facility, University of Texas Southwestern Medical Center, Dallas, TX 75235, USALuciano Merlini - Istituto Ortopedico Rizzoli, Bologna, ItalyKemal Azibi - Hopital Bologhine, CHU, Alger-Ouest, AlgeriaMalika Chaouch - Hopital Ben-Aknoun, CHU, Alger-Ouest, AlgeriaClive Slaughter - Howard Hughes Medical Institute, Biopolymer Facility, University of Texas Southwestern Medical Center, Dallas, TX 75235, USAMichel Fardeau - INSERM 153, 17 rue du Fer à Moulin, 75005 Paris, FranceJean-Claude Kaplan - Laboratoire de Biochimie Génétique et INSERM 129, CHU Cochin, Université René Descartes, 75014 Paris, FranceKevin P Campbell - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USA
- Resource Type
- Journal article
- Publication Details
- FEBS letters, Vol.381(1), pp.15-20
- DOI
- 10.1016/0014-5793(96)00056-7
- PMID
- 8641426
- NLM abbreviation
- FEBS Lett
- ISSN
- 0014-5793
- eISSN
- 1873-3468
- Publisher
- Elsevier B.V
- Language
- English
- Date published
- 1996
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984068389302771
Metrics
19 Record Views