Characterization of δ-sarcoglycan, a novel component of the oligomeric sarcoglycan complex involved in limb-girdle muscular dystrophy

Daniel Jung, Franck Duclos, Barbara Apostol, Volker Straub, Jane C. Lee, Valérie Allamand, David P. Venzke, Yoshihide Sunada, Carolyn R. Moomaw, Cynthia J. Leveille, Clive A. Slaughter, Thomas O. Crawford, John Douglas Mcpherson, Kevin P. Campbell

Research output: Contribution to journalArticle

79 Scopus citations

Abstract

The sarcoglycan complex is known to be involved in limb-girdle muscular dystrophy (LGMD) and is composed of at least three proteins: α-, β-, and γ-sarcoglycan. δ-Sarcoglycan has now been identified as a second 35-kDa sarcolemmal transmembrane glycoprotein that shares high homology with γ- sarcoglycan and is expressed mainly in skeletal and cardiac muscle. Biochemical analysis has demonstrated that γ- and δ-sarcoglycan are separate entities within the sarcoglycan complex and that all four sarcoglycans exist in the complex on a stoichiometrically equal basis. Immunohistochemical analysis of skeletal muscle biopsies from patients with LGMD2C, LGMD2D, and LGMD2E demonstrated a reduction of the entire sarcoglycan complex in these muscular dystrophies. Furthermore, we have mapped the human δ-sarcoglycan gene to chromosome 5q33-q34 in a region overlapping the recently linked autosomal recessive LGMD2F locus.

Original languageEnglish (US)
Pages (from-to)32321-32329
Number of pages9
JournalJournal of Biological Chemistry
Volume271
Issue number50
DOIs
StatePublished - 1996
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry

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    Jung, D., Duclos, F., Apostol, B., Straub, V., Lee, J. C., Allamand, V., Venzke, D. P., Sunada, Y., Moomaw, C. R., Leveille, C. J., Slaughter, C. A., Crawford, T. O., Mcpherson, J. D., & Campbell, K. P. (1996). Characterization of δ-sarcoglycan, a novel component of the oligomeric sarcoglycan complex involved in limb-girdle muscular dystrophy. Journal of Biological Chemistry, 271(50), 32321-32329. https://doi.org/10.1074/jbc.271.50.32321