This Article Order Full text via Infotrieve Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mizuno, Y. Articles by Ozawa, E. Search for Related Content PubMed PubMed Citation Articles by Mizuno, Y. Articles by Ozawa, E.

American Journal of Pathology, Vol 146, 530-536, Copyright © 1995 by American Society for Investigative Pathology

REGULAR ARTICLES

Sarcoglycan complex is selectively lost in dystrophic hamster muscle

Y Mizuno, S Noguchi, H Yamamoto, M Yoshida, I Nonaka, S Hirai and E Ozawa
National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan.

We recently reported that the dystrophin-associated glycoprotein (DAG) complex is biochemically divided into two subcomplexes: one is the dystroglycan complex comprised of 156DAG and 43DAG and the other is the sarcoglycan complex comprised of 50DAG, A3b, and 35DAG. A3b is a novel dystrophin-associated glycoprotein with an approximate molecular mass of 43 kd but is distinct from 43DAG. In the present study, we examined the striated muscles of the dystrophic hamster with anti-A3b antibody in addition to anti-50DAG, anti-43DAG, anti-35DAG, anti-dystrophin, and anti-laminin antibodies by both immunohistochemistry and immunoblot analysis and found that 50DAG, A3b, and 35DAG are selectively lost. This selective defect of the sarcoglycan complex in dystrophic hamster muscles may give rise to dystrophic changes in striated muscles. Thus, the differentiation of the dystrophin-associated glycoprotein complex into the dystroglycan and sarcoglycan complexes is important not only from a biochemical standpoint but also in understanding the cause of muscular dystrophy in the hamster. Our findings further show that the dystrophic hamster may serve as an animal model for a human disease, severe childhood autosomal recessive muscular dystrophy, which has recently been shown to result from a selective defect in the sarcoglycan complex.

This article has been cited by other articles:

				R. H. CROSBIE, R. BARRESI, and K. P. CAMPBELL Loss of sarcolemma nNOS in sarcoglycan-deficient muscle FASEB J, November 1, 2002; 16(13): 1786 - 1791. [Abstract] [Full Text] [PDF]

				R. H. CROSBIE, S. A. DOVICO, J. D. FLANAGAN, J. S. CHAMBERLAIN, C. L. OWNBY, and K. P. CAMPBELL Characterization of aquaporin-4 in muscle and muscular dystrophy FASEB J, July 1, 2002; 16(9): 943 - 949. [Abstract] [Full Text] [PDF]

				Y.-W. Chen, P. Zhao, R. Borup, and E. P. Hoffman Expression Profiling in the Muscular Dystrophies: Identification of Novel Aspects of Molecular Pathophysiology J. Cell Biol., December 11, 2000; 151(6): 1321 - 1336. [Abstract] [Full Text] [PDF]

				X. Xiao, J. Li, Y.-P. Tsao, D. Dressman, E. P. Hoffman, and J. F. Watchko Full Functional Rescue of a Complete Muscle (TA) in Dystrophic Hamsters by Adeno-Associated Virus Vector-Directed Gene Therapy J. Virol., February 1, 2000; 74(3): 1436 - 1442. [Abstract] [Full Text]

				V. Straub, A. J. Ettinger, M. Durbeej, D. P. Venzke, S. Cutshall, J. R. Sanes, and K. P. Campbell epsilon -Sarcoglycan Replaces alpha -Sarcoglycan in Smooth Muscle to Form a Unique Dystrophin-Glycoprotein Complex J. Biol. Chem., September 24, 1999; 274(39): 27989 - 27996. [Abstract] [Full Text] [PDF]

				K. Araishi, T. Sasaoka, M. Imamura, S. Noguchi, H. Hama, E. Wakabayashi, M. Yoshida, T. Hori, and E. Ozawa Loss of the sarcoglycan complex and sarcospan leads to muscular dystrophy in {beta}-sarcoglycan-deficient mice Hum. Mol. Genet., September 1, 1999; 8(9): 1589 - 1598. [Abstract] [Full Text] [PDF]

				R. H. Crosbie, C. S. Lebakken, K. H. Holt, D. P. Venzke, V. Straub, J. C. Lee, R. M. Grady, J. S. Chamberlain, J. R. Sanes, and K. P. Campbell Membrane Targeting and Stabilization of Sarcospan Is Mediated by the Sarcoglycan Subcomplex J. Cell Biol., April 5, 1999; 145(1): 153 - 165. [Abstract] [Full Text] [PDF]

				K. H. Holt and K. P. Campbell Assembly of the Sarcoglycan Complex. INSIGHTS FOR MUSCULAR DYSTROPHY J. Biol. Chem., December 25, 1998; 273(52): 34667 - 34670. [Abstract] [Full Text] [PDF]

				V. Straub, F. Duclos, D. P. Venzke, J. C. Lee, S. Cutshall, C. J. Leveille, and K. P. Campbell Molecular Pathogenesis of Muscle Degeneration in the {delta}-Sarcoglycan-Deficient Hamster Am. J. Pathol., November 1, 1998; 153(5): 1623 - 1630. [Abstract] [Full Text] [PDF]