The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

SGCB  -  sarcoglycan, beta (43kDa dystrophin...

Homo sapiens

Synonyms: 43 kDa dystrophin-associated glycoprotein, 43DAG, A3b, Beta-SG, Beta-sarcoglycan, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of SGCB


High impact information on SGCB


Biological context of SGCB


Anatomical context of SGCB


Associations of SGCB with chemical compounds

  • The patient's sample showed a band of increased mobility in exon 4 of the beta-sarcoglycan gene which, upon sequencing, was found to represent a homozygous A-->G transversion at nucleotide 551, resulting in a tyrosine to cysteine substitution at position 184 (Y184C) [10].
  • Multiple ligand co-recognition of 3'-sulfogalactosylceramide (SGC) and sulfotyrosine initiated the comparison of SGC and sulfotyrosine and, subsequently, phosphotyrosine (pY) binding [18].
  • A variety of natural glycosphingolipids (GSLs): GlcC, GalC, SGC, LC, Gb3C, Gb4C, Gg4C, Gb5C, and GM1C, gave the corresponding acids: 2-hydroxy-3-(N-acyl)-4-(O-glycosyl)-oxybutyric acids, i.e. "glycosyl ceramide acids" (GSL.CCOOH) in excellent yields (80-90%) [4].
  • Using hsp70, anti-SGC, and anti-pY antibodies, ligand binding is retained following phosphate/sulfate and tyrosine/galactose substitution in SGC and sulfate/phosphate exchange in pY [18].
  • In addition, in teased nerve preparations, mAb 224-58-positive cells were also galactosylceramide (GalC)- and SGC-positive [19].

Physical interactions of SGCB


Other interactions of SGCB


Analytical, diagnostic and therapeutic context of SGCB

  • 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 [23].
  • We show by immunofluorescence and Western blotting that in skeletal muscle from these patients gamma-sarcoglycan is completely absent and alpha- and beta-sarcoglycan are greatly reduced in abundance, whereas other components of the DGC are preserved [25].
  • Advanced light-microscopic imaging techniques demonstrated that co-expression of beta-sarcoglycan and delta-sarcoglycan is also responsible for delivery to and retention of sarcoglycan subcomplexes at the cell surface [26].
  • We have analyzed the distribution of the five tetrameric Bandeiraea simplicifolia I isolectins (A4, A3b, A2B2, AB3, B4) isolated by melibionate Bio-Gel affinity chromatography from 168 single seeds gathered in Ghana from known trees and pods [27].
  • This suggests that although 224-58 does not discriminate between SGC and seminolipid in serological tests, these lipids in their naturally occurring membrane acquire a spatial configuration that renders them distinguishable to their respective antibody [19].


  1. Beta-sarcoglycan (A3b) mutations cause autosomal recessive muscular dystrophy with loss of the sarcoglycan complex. Bönnemann, C.G., Modi, R., Noguchi, S., Mizuno, Y., Yoshida, M., Gussoni, E., McNally, E.M., Duggan, D.J., Angelini, C., Hoffman, E.P. Nat. Genet. (1995) [Pubmed]
  2. Beta-sarcoglycan: characterization and role in limb-girdle muscular dystrophy linked to 4q12. Lim, L.E., Duclos, F., Broux, O., Bourg, N., Sunada, Y., Allamand, V., Meyer, J., Richard, I., Moomaw, C., Slaughter, C. Nat. Genet. (1995) [Pubmed]
  3. Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E. Durbeej, M., Cohn, R.D., Hrstka, R.F., Moore, S.A., Allamand, V., Davidson, B.L., Williamson, R.A., Campbell, K.P. Mol. Cell (2000) [Pubmed]
  4. A convenient oxidation of natural glycosphingolipids to their "ceramide acids" for neoglycoconjugation. Bovine serum albumin-glycosylceramide acid conjugates as investigative probes for HIV gp120 coat protein-glycosphingolipid interactions. Mylvaganam, M., Lingwood, C.A. J. Biol. Chem. (1999) [Pubmed]
  5. Immunogold localization of the 43-kDa dystroglycan at the plasma membrane in control and dystrophic human muscle. Cullen, M.J., Walsh, J., Nicholson, L.V. Acta Neuropathol. (1994) [Pubmed]
  6. Mutations in the sarcoglycan genes in patients with myopathy. Duggan, D.J., Gorospe, J.R., Fanin, M., Hoffman, E.P., Angelini, C. N. Engl. J. Med. (1997) [Pubmed]
  7. Loss of the sarcoglycan complex and sarcospan leads to muscular dystrophy in beta-sarcoglycan-deficient mice. Araishi, K., Sasaoka, T., Imamura, M., Noguchi, S., Hama, H., Wakabayashi, E., Yoshida, M., Hori, T., Ozawa, E. Hum. Mol. Genet. (1999) [Pubmed]
  8. Genomic screening for beta-sarcoglycan gene mutations: missense mutations may cause severe limb-girdle muscular dystrophy type 2E (LGMD 2E). Bönnemann, C.G., Passos-Bueno, M.R., McNally, E.M., Vainzof, M., de Sá Moreira, E., Marie, S.K., Pavanello, R.C., Noguchi, S., Ozawa, E., Zatz, M., Kunkel, L.M. Hum. Mol. Genet. (1996) [Pubmed]
  9. Overexpression of gamma-sarcoglycan induces severe muscular dystrophy. Implications for the regulation of Sarcoglycan assembly. Zhu, X., Hadhazy, M., Groh, M.E., Wheeler, M.T., Wollmann, R., McNally, E.M. J. Biol. Chem. (2001) [Pubmed]
  10. Noval mutation (Y184C) in exon 4 of the beta-sarcoglycan gene identified in a Portuguese patient. Mutations in brief no. 177. Online. dos Santos, M.R., Jorge, P., Ribeiro, E.M., Pires, M.M., Guimarães, A. Hum. Mutat. (1998) [Pubmed]
  11. Private beta- and gamma-sarcoglycan gene mutations: evidence of a founder effect in Northern Italy. Fanin, M., Hoffman, E.P., Angelini, C., Pegoraro, E. Hum. Mutat. (2000) [Pubmed]
  12. A new evidence for the maintenance of the sarcoglycan complex in muscle sarcolemma in spite of the primary absence of delta-SG protein. Gouveia, T.L., Kossugue, P.M., Paim, J.F., Zatz, M., Anderson, L.V., Nigro, V., Vainzof, M. J. Mol. Med. (2007) [Pubmed]
  13. Characterization of delta-sarcoglycan, a novel component of the oligomeric sarcoglycan complex involved in limb-girdle muscular dystrophy. 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. J. Biol. Chem. (1996) [Pubmed]
  14. Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in alpha- and gamma-sarcoglycanopathy: an immunohistochemical study. Anastasi, G., Cutroneo, G., Trimarchi, F., Santoro, G., Bruschetta, D., Bramanti, P., Pisani, A., Favaloro, A. Int. J. Mol. Med. (2004) [Pubmed]
  15. Confocal analysis of the dystrophin protein complex in muscular dystrophy. Draviam, R., Billington, L., Senchak, A., Hoffman, E.P., Watkins, S.C. Muscle Nerve (2001) [Pubmed]
  16. Defective assembly of sarcoglycan complex in patients with beta-sarcoglycan gene mutations. Study of aneural and innervated cultured myotubes. Fanin, M., Angelini, C. Neuropathol. Appl. Neurobiol. (2002) [Pubmed]
  17. Distribution of dystrophin isoforms and dystrophin-associated proteins 43DAG (A3a) and 50DAG (A2) in various monkey tissues. Mizuno, Y., Yoshida, M., Yamamoto, H., Hirai, S., Ozawa, E. J. Biochem. (1993) [Pubmed]
  18. The sulfogalactose moiety of sulfoglycosphingolipids serves as a mimic of tyrosine phosphate in many recognition processes. Prediction and demonstration of Src homology 2 domain/sulfogalactose binding. Lingwood, C., Mylvaganam, M., Minhas, F., Binnington, B., Branch, D.R., Pomès, R. J. Biol. Chem. (2005) [Pubmed]
  19. Schwann cell marker defined by a monoclonal antibody (224-58) with species cross-reactivity. I. Cellular localization. Guerci, A., Monge, M., Baron-Van Evercooren, A., Lubetzki, C., Dancea, S., Boutry, J.M., Goujet-Zalc, C., Zalc, B. J. Neurochem. (1986) [Pubmed]
  20. Molecular organization at the glycoprotein-complex-binding site of dystrophin. Three dystrophin-associated proteins bind directly to the carboxy-terminal portion of dystrophin. Suzuki, A., Yoshida, M., Hayashi, K., Mizuno, Y., Hagiwara, Y., Ozawa, E. Eur. J. Biochem. (1994) [Pubmed]
  21. Autosomal recessive limb-girdle muscular dystrophy, LGMD2F, is caused by a mutation in the delta-sarcoglycan gene. Nigro, V., de Sá Moreira, E., Piluso, G., Vainzof, M., Belsito, A., Politano, L., Puca, A.A., Passos-Bueno, M.R., Zatz, M. Nat. Genet. (1996) [Pubmed]
  22. Beta-sarcoglycan gene mutations in Turkey. Balci, B., Wilichowski, E., Haliloğlu, G., Talim, B., Aurino, S., Kremer, E., Ebinger, F., Senbil, N., Anlar, B., Kale, G., Nigro, V., Topaloğlu, H., Bonnemann, C., Dinçer, P. Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology / edited by the Gaetano Conte Academy for the study of striated muscle diseases. (2004) [Pubmed]
  23. Sarcoglycan complex is selectively lost in dystrophic hamster muscle. Mizuno, Y., Noguchi, S., Yamamoto, H., Yoshida, M., Nonaka, I., Hirai, S., Ozawa, E. Am. J. Pathol. (1995) [Pubmed]
  24. Genetic epidemiology of muscular dystrophies resulting from sarcoglycan gene mutations. Fanin, M., Duggan, D.J., Mostacciuolo, M.L., Martinello, F., Freda, M.P., Sorarù, G., Trevisan, C.P., Hoffman, E.P., Angelini, C. J. Med. Genet. (1997) [Pubmed]
  25. Absence of gamma-sarcoglycan (35 DAG) in autosomal recessive muscular dystrophy linked to chromosome 13q12. Jung, D., Leturcq, F., Sunada, Y., Duclos, F., Tomé, F.M., Moomaw, C., Merlini, L., Azibi, K., Chaouch, M., Slaughter, C., Fardeau, M., Kaplan, J.C., Campbell, K.P. FEBS Lett. (1996) [Pubmed]
  26. The beta-delta-core of sarcoglycan is essential for deposition at the plasma membrane. Draviam, R.A., Shand, S.H., Watkins, S.C. Muscle Nerve (2006) [Pubmed]
  27. Bandeiraea simplicifolia I isolectins reveal a development sequential relationship. Lamb, J.E., Bookstein, F.L., Goldstein, I.J., Newton, L.E. J. Biol. Chem. (1981) [Pubmed]
WikiGenes - Universities