Sgcg

Summary

Gene Symbol: Sgcg
Description: sarcoglycan, gamma (dystrophin-associated glycoprotein)
Alias: 35kDa, 5430420E18Rik, AI642964, gamma-SG, gamma-sarcoglycan, 35 kDa dystrophin-associated glycoprotein, 35DAG, sarcoglycan, gamma (35kD dystrophin-associated glycoprotein)
Species: mouse
Products:     Sgcg

Top Publications

  1. Griffin M, Feng H, Tewari M, Acosta P, Kawana M, Sweeney H, et al. gamma-Sarcoglycan deficiency increases cell contractility, apoptosis and MAPK pathway activation but does not affect adhesion. J Cell Sci. 2005;118:1405-16 pubmed
    ..We conclude that gammaSG normally moderates contractile prestress in skeletal muscle, and we propose a role for gammaSG in membrane-based signaling of the effects of prestress and sarcomerogenesis. ..
  2. Hack A, Ly C, Jiang F, Clendenin C, Sigrist K, Wollmann R, et al. Gamma-sarcoglycan deficiency leads to muscle membrane defects and apoptosis independent of dystrophin. J Cell Biol. 1998;142:1279-87 pubmed
    ..As a common molecular feature in a variety of muscular dystrophies, sarcoglycan loss is a likely mediator of pathology. ..
  3. Heydemann A, Ceco E, Lim J, Hadhazy M, Ryder P, Moran J, et al. Latent TGF-beta-binding protein 4 modifies muscular dystrophy in mice. J Clin Invest. 2009;119:3703-12 pubmed publisher
    ..In contrast, a 12-amino-acid deletion in LTBP4 was associated with increased proteolysis, SMAD signaling, and fibrosis. These data identify Ltbp4 as a target gene to regulate TGF-beta signaling and modify outcomes in muscular dystrophy. ..
  4. Chan Y, Bonnemann C, Lidov H, Kunkel L. Molecular organization of sarcoglycan complex in mouse myotubes in culture. J Cell Biol. 1998;143:2033-44 pubmed
  5. Allikian M, Hack A, Mewborn S, Mayer U, McNally E. Genetic compensation for sarcoglycan loss by integrin alpha7beta1 in muscle. J Cell Sci. 2004;117:3821-30 pubmed
  6. Goldstein J, Bogdanovich S, Beiriger A, Wren L, Rossi A, Gao Q, et al. Excess SMAD signaling contributes to heart and muscle dysfunction in muscular dystrophy. Hum Mol Genet. 2014;23:6722-31 pubmed publisher
    ..Muscle lacking the dystrophin-associated protein γ-sarcoglycan (Sgcg null) was subjected to a lengthening protocol to produce maximal muscle injury, which produced rapid accumulation ..
  7. Heydemann A, Huber J, Demonbreun A, Hadhazy M, McNally E. Genetic background influences muscular dystrophy. Neuromuscul Disord. 2005;15:601-9 pubmed
    ..Identification of these modifier genes and the associated pathways may lead to novel therapeutic strategies. ..
  8. Noguchi S, Wakabayashi Takai E, Sasaoka T, Ozawa E. Analysis of the spatial, temporal and tissue-specific transcription of gamma-sarcoglycan gene using a transgenic mouse. FEBS Lett. 2001;495:77-81 pubmed
  9. Wheeler M, Zarnegar S, McNally E. Zeta-sarcoglycan, a novel component of the sarcoglycan complex, is reduced in muscular dystrophy. Hum Mol Genet. 2002;11:2147-54 pubmed
    ..Together, these data demonstrate that zeta-sarcoglycan is an integral component of the sarcoglycan complex and, as such, is important in the pathogenesis of muscular dystrophy. ..

More Information

Publications43

  1. Juo L, Liao W, Shih Y, Yang B, Liu A, Yan Y. HSPB7 interacts with dimerized FLNC and its absence results in progressive myopathy in skeletal muscles. J Cell Sci. 2016;129:1661-70 pubmed publisher
    ..Collectively, our findings suggest that HSPB7 is essential for maintaining muscle integrity, which is achieved through its interaction with FLNC, in order to prevent the occurrence and progression of myopathy. ..
  2. Nguyen A, Xiao B, Neppl R, Kallin E, Li J, Chen T, et al. DOT1L regulates dystrophin expression and is critical for cardiac function. Genes Dev. 2011;25:263-74 pubmed publisher
    ..In addition, our study may open new avenues for the diagnosis and treatment of human heart disease. ..
  3. Wheeler M, Korcarz C, Collins K, Lapidos K, Hack A, Lyons M, et al. Secondary coronary artery vasospasm promotes cardiomyopathy progression. Am J Pathol. 2004;164:1063-71 pubmed
    ..30 ml/minute/g versus 0.67 +/- 0.16 ml/minute/g, P < 0.05). These data indicate that secondary vasospasm contributes to the development of cardiomyopathy and is an important therapeutic target to limit cardiomyopathy progression. ..
  4. Quattrocelli M, Salamone I, Page P, Warner J, Demonbreun A, McNally E. Intermittent Glucocorticoid Dosing Improves Muscle Repair and Function in Mice with Limb-Girdle Muscular Dystrophy. Am J Pathol. 2017;187:2520-2535 pubmed publisher
    ..These data indicate that dosing frequency of glucocorticoid steroids affects muscle remodeling in non-Duchenne muscular dystrophies, suggesting a positive outcome associated with intermittent steroid dosing in LGMD 2B and 2C muscle. ..
  5. Fukai Y, Ohsawa Y, Ohtsubo H, Nishimatsu S, Hagiwara H, Noda M, et al. Cleavage of ?-dystroglycan occurs in sarcoglycan-deficient skeletal muscle without MMP-2 and MMP-9. Biochem Biophys Res Commun. 2017;492:199-205 pubmed publisher
    ..Thus, matrix metalloproteinases contributing to ?-dystroglycan cleavage are redundant, and MMP-14 could participate in the pathogenesis of sarcoglycanopathy. ..
  6. Roberts N, Holley Cuthrell J, Gonzalez Vega M, Mull A, Heydemann A. Biochemical and Functional Comparisons of mdx and Sgcg(-/-) Muscular Dystrophy Mouse Models. Biomed Res Int. 2015;2015:131436 pubmed publisher
    ..now comparing disease progression from one of these alternative gene disruptions, the γ-sarcoglycan null mouse Sgcg(-/-) on the DBA2/J background, to the mdx mouse line...
  7. Heydemann A, Huber J, Kakkar R, Wheeler M, McNally E. Functional nitric oxide synthase mislocalization in cardiomyopathy. J Mol Cell Cardiol. 2004;36:213-23 pubmed
    ..These data provide a mechanism where regional, focal cardiac damage creates pathologic gradients of NO. Moreover, inhibition of nitric oxide synthase corrects defects that arise from pathologic NO gradients. ..
  8. Hack A, Lam M, Cordier L, Shoturma D, Ly C, Hadhazy M, et al. Differential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin-glycoprotein complex. J Cell Sci. 2000;113 ( Pt 14):2535-44 pubmed
    ..Furthermore, these molecular differences were associated with different mechanical consequences for the muscle plasma membrane. Through this in vivo analysis, a model for sarcoglycan assembly is proposed. ..
  9. Straub V, Ettinger A, Durbeej M, Venzke D, Cutshall S, Sanes J, et al. epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex. J Biol Chem. 1999;274:27989-96 pubmed
    ..Our results also suggest a molecular basis for possible differential smooth muscle dysfunction in sarcoglycan-deficient patients. ..
  10. Chen J, Skinner M, Shi W, Yu Q, Wildeman A, Chan Y. The 16 kDa subunit of vacuolar H+-ATPase is a novel sarcoglycan-interacting protein. Biochim Biophys Acta. 2007;1772:570-9 pubmed
  11. Zhu X, Wheeler M, Hadhazy M, Lam M, McNally E. Cardiomyopathy is independent of skeletal muscle disease in muscular dystrophy. FASEB J. 2002;16:1096-8 pubmed
  12. Swaggart K, Heydemann A, Palmer A, McNally E. Distinct genetic regions modify specific muscle groups in muscular dystrophy. Physiol Genomics. 2011;43:24-31 pubmed publisher
    ..Using the Sgcg model of limb girdle muscular dystrophy that lacks the dystrophin-associated protein ?-sarcoglycan, we evaluated ..
  13. Turk R, Sterrenburg E, van der Wees C, de Meijer E, de Menezes R, Groh S, et al. Common pathological mechanisms in mouse models for muscular dystrophies. FASEB J. 2006;20:127-9 pubmed
    ..of the following mouse models: dystrophin-deficient (mdx, mdx(3cv)), sarcoglycan-deficient (Sgca null, Sgcb null, Sgcg null, Sgcd null), dysferlin-deficient (Dysf null, SJL(Dysf)), sarcospan-deficient (Sspn null), and wild-type (C57Bl/..
  14. Chen J, Shi W, Zhang Y, Sokol R, Cai H, Lun M, et al. Identification of functional domains in sarcoglycans essential for their interaction and plasma membrane targeting. Exp Cell Res. 2006;312:1610-25 pubmed
    ..Our results therefore generate important information on the structure of the sarcoglycan complex and the molecular mechanisms underlying the effects of various sarcoglycan mutations in muscular dystrophies. ..
  15. Heydemann A, Doherty K, McNally E. Genetic modifiers of muscular dystrophy: implications for therapy. Biochim Biophys Acta. 2007;1772:216-28 pubmed
    ..The value of these genes and products is that the pathways identified through these experiments may be exploited for therapy. ..
  16. Swaggart K, Demonbreun A, Vo A, Swanson K, Kim E, Fahrenbach J, et al. Annexin A6 modifies muscular dystrophy by mediating sarcolemmal repair. Proc Natl Acad Sci U S A. 2014;111:6004-9 pubmed publisher
    ..In contrast, ANXA6N32 dramatically disrupted the annexin A6-rich cap and the associated repair zone, permitting membrane leak. Anxa6 is a modifier of muscular dystrophy and membrane repair after injury. ..
  17. Rouillon J, Poupiot J, Zocevic A, Amor F, Léger T, García C, et al. Serum proteomic profiling reveals fragments of MYOM3 as potential biomarkers for monitoring the outcome of therapeutic interventions in muscular dystrophies. Hum Mol Genet. 2015;24:4916-32 pubmed publisher
    ..These data suggest that the MYOM3 fragments hold promise for minimally invasive assessment of experimental therapies for DMD and other neuromuscular disorders. ..
  18. Barton E. Restoration of gamma-sarcoglycan localization and mechanical signal transduction are independent in murine skeletal muscle. J Biol Chem. 2010;285:17263-70 pubmed publisher
    ..This study shows that localization of gamma-SG does not require Tyr(6), but localization alone is insufficient for restoration of normal signal transduction patterns after mechanical perturbation. ..
  19. Gao Q, Wyatt E, Goldstein J, LOPRESTI P, Castillo L, Gazda A, et al. Reengineering a transmembrane protein to treat muscular dystrophy using exon skipping. J Clin Invest. 2015;125:4186-95 pubmed publisher
    ..Since Mini-Gamma represents removal of 4 of the 7 coding exons in γ-sarcoglycan, this approach provides a viable strategy to treat the majority of patients with γ-sarcoglycan gene mutations. ..
  20. Hack A, Cordier L, Shoturma D, Lam M, Sweeney H, McNally E. Muscle degeneration without mechanical injury in sarcoglycan deficiency. Proc Natl Acad Sci U S A. 1999;96:10723-8 pubmed
    ..Thus, a nonmechanical mechanism, perhaps involving some unknown signaling function, likely is responsible for muscular dystrophy where sarcoglycan is deficient. ..
  21. Sasaoka T, Imamura M, Araishi K, Noguchi S, Mizuno Y, Takagoshi N, et al. Pathological analysis of muscle hypertrophy and degeneration in muscular dystrophy in gamma-sarcoglycan-deficient mice. Neuromuscul Disord. 2003;13:193-206 pubmed
    ..The muscle pathology became more 'dystrophic' in mice over 1 year of age when there was a marked variation in fiber size with interstitial fibrosis. ..
  22. Barresi R, Moore S, Stolle C, Mendell J, Campbell K. Expression of gamma -sarcoglycan in smooth muscle and its interaction with the smooth muscle sarcoglycan-sarcospan complex. J Biol Chem. 2000;275:38554-60 pubmed
    ..Taken together, our results prove that the sarcoglycan complex in vascular and visceral smooth muscle consists of epsilon-, beta-, gamma-, and delta-sarcoglycans and is associated with sarcospan. ..
  23. Johnson E, Zhang L, Adams M, Phillips A, Freitas M, Froehner S, et al. Proteomic analysis reveals new cardiac-specific dystrophin-associated proteins. PLoS ONE. 2012;7:e43515 pubmed publisher
    ..In addition, our findings support the existence of cardiac-specific functions of dystrophin and may guide studies into early triggers of cardiac disease in Duchenne and Becker muscular dystrophies. ..
  24. Townsend D, Yasuda S, McNally E, Metzger J. Distinct pathophysiological mechanisms of cardiomyopathy in hearts lacking dystrophin or the sarcoglycan complex. FASEB J. 2011;25:3106-14 pubmed publisher
  25. Gastaldello S, D Angelo S, Franzoso S, Fanin M, Angelini C, Betto R, et al. Inhibition of proteasome activity promotes the correct localization of disease-causing alpha-sarcoglycan mutants in HEK-293 cells constitutively expressing beta-, gamma-, and delta-sarcoglycan. Am J Pathol. 2008;173:170-81 pubmed publisher
    ..These data provide important insights for the potential development of pharmacological therapies for sarcoglycanopathies. ..
  26. Spinazzola J, Smith T, Liu M, Luna E, Barton E. Gamma-sarcoglycan is required for the response of archvillin to mechanical stimulation in skeletal muscle. Hum Mol Genet. 2015;24:2470-81 pubmed publisher
    ..These results position archvillin as a mechanically sensitive component of the dystrophin complex and demonstrate that signaling defects caused by loss of γ-SG occur both at the sarcolemma and in the nucleus. ..
  27. Wheeler M, Allikian M, Heydemann A, Hadhazy M, Zarnegar S, McNally E. Smooth muscle cell-extrinsic vascular spasm arises from cardiomyocyte degeneration in sarcoglycan-deficient cardiomyopathy. J Clin Invest. 2004;113:668-75 pubmed
    ..Therefore, we propose that cytokine release from damaged cardiomyocytes can feed back to produce vascular spasm. Moreover, vascular spasm feeds forward to produce additional cardiac damage...
  28. Goldstein J, Kelly S, LoPresti P, Heydemann A, Earley J, Ferguson E, et al. SMAD signaling drives heart and muscle dysfunction in a Drosophila model of muscular dystrophy. Hum Mol Genet. 2011;20:894-904 pubmed publisher
    ..Reduction of this pathway is sufficient to restore cardiac and muscle function and is therefore a target for therapeutic reduction. ..
  29. Noguchi S, Wakabayashi E, Imamura M, Yoshida M, Ozawa E. Developmental expression of sarcoglycan gene products in cultured myocytes. Biochem Biophys Res Commun. 1999;262:88-93 pubmed
    ..The discrepancy between the expression of the mRNAs and proteins of the sarcoglycan subunits in proliferating cells may be ascribed to rapid degradation of the protein. ..
  30. Durbeej M, Cohn R, Hrstka R, Moore S, Allamand V, Davidson B, et al. Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E. Mol Cell. 2000;5:141-51 pubmed
    ..Thus, perturbation of vascular function together with disruption of the epsilon-sarcoglycan-containing complex represents a novel mechanism in the pathogenesis of LGMD 2E. ..
  31. Vignier N, Amor F, Fogel P, Duvallet A, Poupiot J, Charrier S, et al. Distinctive serum miRNA profile in mouse models of striated muscular pathologies. PLoS ONE. 2013;8:e55281 pubmed publisher
    ..The differential expression of a few dysregulated miRNAs in the DMD mice was further evaluated in DMD patients, providing new candidates of circulating miRNA biomarkers for DMD. ..
  32. Porter J, Merriam A, Hack A, Andrade F, McNally E. Extraocular muscle is spared despite the absence of an intact sarcoglycan complex in gamma- or delta-sarcoglycan-deficient mice. Neuromuscul Disord. 2001;11:197-207 pubmed
  33. Barton E. Impact of sarcoglycan complex on mechanical signal transduction in murine skeletal muscle. Am J Physiol Cell Physiol. 2006;290:C411-9 pubmed
    ..This study provides evidence that the SGs are involved in the transduction of mechanical information in skeletal muscle, potentially unique from the entire DGC...
  34. Madhavan R, Jarrett H. Interactions between dystrophin glycoprotein complex proteins. Biochemistry. 1995;34:12204-9 pubmed
    ..The interactions identified here were used to refine the existing model of DGC organization to make it consistent with the current data. ..