JBC

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Liu, L. A.
Right arrow Articles by Engvall, E.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Liu, L. A.
Right arrow Articles by Engvall, E.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?

J Biol Chem, Vol. 274, Issue 53, 38171-38176, December 31, 1999

Sarcoglycan Isoforms in Skeletal Muscle*

Ling A. LiuDagger and Eva EngvallDagger §

From the Dagger  Burnham Institute, La Jolla, California 92037 and the § Department of Developmental Biology, Wenner-Gren Institute, Stockholm University, S-10691 Stockholm, Sweden

The heterotetrameric sarcoglycan complex, composed of alpha -, beta -, gamma -, and delta -sarcoglycans, is an important component of the dystrophin-associated glycoprotein assembly in striated muscle. Mutations in any of the four genes encoding sarcoglycans cause a deficiency in all sarcoglycans in the sarcolemma and produce one of four types of limb-girdle muscular dystrophy. A fifth widely expressed sarcoglycan, epsilon -sarcoglycan, has been recently described. epsilon -Sarcoglycan is homologous to alpha -sarcoglycan, but whether it associates with the other sarcoglycans in muscle is not known. In this study, we use wild type and alpha -sarcoglycan-deficient mice to analyze the localization and association of sarcoglycans in skeletal muscle in vivo. The amounts of beta -, gamma -, and delta -sarcoglycans are reduced in alpha -sarcoglycan mutants, whereas the amount of epsilon -sarcoglycan is unchanged. We show here that epsilon -sarcoglycan is complexed with beta -, gamma -, and delta -sarcoglycans in both wild type and alpha -sarcoglycan mutant mice. We also use C2C12 myocytes to study the temporal expression and organization of sarcoglycan complexes during muscle cell differentiation in vitro. In C2C12 cells, alpha - and epsilon -sarcoglycans form separate complexes with beta -, gamma -, and delta -sarcoglycans. Both types of complexes are expressed at the cell surface and presumed to be functional. These results suggest that epsilon -sarcoglycan serves a function similar to that of alpha -sarcoglycan and that residual beta -, gamma -, and delta -sarcoglycan seen in mutant mice and alpha -sarcoglycan-deficient patients is due to its association with epsilon -sarcoglycan.

* This work was supported in part by grants from the National Institutes of Health and the Muscular Dystrophy Association.The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

To whom correspondence should be addressed: Burnham Inst., 10901 N. Torrey Pines Rd., La Jolla, CA 92037. Tel.: 858-646-3100; Fax: 858-646-3199; E-mail: eengvall@burnham.org.

Copyright © 1999 by The American Society for Biochemistry and Molecular Biology, Inc.
Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
 J. Biol. Chem.Home page
L. A. Cunningham and R. A. Kahn
Cofactor D Functions as a Centrosomal Protein and Is Required for the Recruitment of the {gamma}-Tubulin Ring Complex at Centrosomes and Organization of the Mitotic Spindle
J. Biol. Chem., March 14, 2008; 283(11): 7155 - 7165.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
M. J. Allikian, G. Bhabha, P. Dospoy, A. Heydemann, P. Ryder, J. U. Earley, M. J. Wolf, H. A. Rockman, and E. M. McNally
Reduced life span with heart and muscle dysfunction in Drosophila sarcoglycan mutants
Hum. Mol. Genet., December 1, 2007; 16(23): 2933 - 2943.
[Abstract] [Full Text] [PDF]

Home page
 J. Histochem. Cytochem.Home page
G. Anastasi, G. Cutroneo, A. Sidoti, C. Rinaldi, D. Bruschetta, G. Rizzo, R. D'Angelo, G. Tarone, A. Amato, and A. Favaloro
Sarcoglycan Subcomplex Expression in Normal Human Smooth Muscle
J. Histochem. Cytochem., August 1, 2007; 55(8): 831 - 843.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
C. T. Esapa, A. Waite, M. Locke, M. A. Benson, M. Kraus, R.A. J. McIlhinney, R. V. Sillitoe, P. W. Beesley, and D. J. Blake
SGCE missense mutations that cause myoclonus-dystonia syndrome impair {varepsilon}-sarcoglycan trafficking to the plasma membrane: modulation by ubiquitination and torsinA
Hum. Mol. Genet., February 1, 2007; 16(3): 327 - 342.
[Abstract] [Full Text] [PDF]

Home page
 J. Gerontol. A Biol. Sci. Med. Sci.Home page
K. M. Rice, D. L. Preston, D. Neff, M. Norton, and E. R. Blough
Age-Related Dystrophin-Glycoprotein Complex Structure and Function in the Rat Extensor Digitorum Longus and Soleus Muscle
J. Gerontol. A Biol. Sci. Med. Sci., November 1, 2006; 61(11): 1119 - 1129.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
S. Homma, M. Iwasaki, G. D. Shelton, E. Engvall, J. C. Reed, and S. Takayama
BAG3 Deficiency Results in Fulminant Myopathy and Early Lethality
Am. J. Pathol., September 1, 2006; 169(3): 761 - 773.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Regul. Integr. Comp. Physiol.Home page
D. Danieli-Betto, A. Esposito, E. Germinario, D. Sandona, T. Martinello, A. Jakubiec-Puka, D. Biral, and R. Betto
Deficiency of {alpha}-sarcoglycan differently affects fast- and slow-twitch skeletal muscles
Am J Physiol Regulatory Integrative Comp Physiol, November 1, 2005; 289(5): R1328 - R1337.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
M. Imamura, Y. Mochizuki, E. Engvall, and S. Takeda
{varepsilon}-Sarcoglycan compensates for lack of {alpha}-sarcoglycan in a mouse model of limb-girdle muscular dystrophy
Hum. Mol. Genet., March 15, 2005; 14(6): 775 - 783.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
K. A. Lapidos, R. Kakkar, and E. M. McNally
The Dystrophin Glycoprotein Complex: Signaling Strength and Integrity for the Sarcolemma
Circ. Res., April 30, 2004; 94(8): 1023 - 1031.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
M. Durbeej, S. M. Sawatzki, R. Barresi, K. M. Schmainda, V. Allamand, D. E. Michele, and K. P. Campbell
Gene transfer establishes primacy of striated vs. smooth muscle sarcoglycan complex in limb-girdle muscular dystrophy
PNAS, July 22, 2003; 100(15): 8910 - 8915.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Cell Physiol.Home page
N. D. Patel, S. R. Jannapureddy, W. Hwang, I. Chaudhry, and A. M. Boriek
Altered muscle force and stiffness of skeletal muscles in alpha -sarcoglycan-deficient mice
Am J Physiol Cell Physiol, April 1, 2003; 284(4): C962 - C968.
[Abstract] [Full Text] [PDF]

Home page
 Journal of the American Animal Hospital AssociationHome page
R. L. Bergman, K. D. Inzana, W. E. Monroe, L. G. Shell, L. A. Liu, E. Engvall, and G. D. Shelton
Dystrophin-Deficient Muscular Dystrophy in a Labrador Retriever
J. Am. Anim. Hosp. Assoc., May 1, 2002; 38(3): 255 - 261.
[Abstract] [Full Text] [PDF]

Home page
 Physiol. Rev.Home page
D. J. Blake, A. Weir, S. E. Newey, and K. E. Davies
Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle
Physiol Rev, April 1, 2002; 82(2): 291 - 329.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
M. Imamura, K. Araishi, S. Noguchi, and E. Ozawa
A sarcoglycan-dystroglycan complex anchors Dp116 and utrophin in the peripheral nervous system
Hum. Mol. Genet., December 1, 2000; 9(20): 3091 - 3100.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
V. Allamand and K. P. Campbell
Animal models for muscular dystrophy: valuable tools for the development of therapies
Hum. Mol. Genet., October 1, 2000; 9(16): 2459 - 2467.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
M.-F. Galliano, C. Huet, J. Frygelius, A. Polgren, U. M. Wewer, and E. Engvall
Binding of ADAM12, a Marker of Skeletal Muscle Regeneration, to the Muscle-specific Actin-binding Protein, alpha -Actinin-2, Is Required for Myoblast Fusion
J. Biol. Chem., April 28, 2000; 275(18): 13933 - 13939.
[Abstract] [Full Text] [PDF]

Home page
 J. Cell Sci.Home page
A. Hack, M. Lam, L Cordier, D. Shoturma, C. Ly, M. Hadhazy, M. Hadhazy, H. Sweeney, and E. McNally
Differential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin-glycoprotein complex
J. Cell Sci., January 7, 2000; 113(14): 2535 - 2544.
[Abstract] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
 All ASBMB Journals   Molecular and Cellular Proteomics 
 Journal of Lipid Research   ASBMB Today 
Copyright © 1999 by the American Society for Biochemistry and Molecular Biology.