Sspn

Summary

Gene Symbol: Sspn
Description: sarcospan
Alias: Krag, sarcospan, K-ras oncogene-associated protein, Kras oncogene-associated, kirsten-Ras-associated protein
Species: mouse
Products:     Sspn

Top Publications

  1. Heighway J, Betticher D, Hoban P, Altermatt H, Cowen R. Coamplification in tumors of KRAS2, type 2 inositol 1,4,5 triphosphate receptor gene, and a novel human gene, KRAG. Genomics. 1996;35:207-14 pubmed
    ..region of DNA, coamplified in tumors with KRAS2, resulted in the identification of the human homologue of the mouse KRAG gene...
  2. Lebakken C, Venzke D, Hrstka R, Consolino C, Faulkner J, Williamson R, et al. Sarcospan-deficient mice maintain normal muscle function. Mol Cell Biol. 2000;20:1669-77 pubmed
    b>Sarcospan is an integral membrane component of the dystrophin-glycoprotein complex (DGC) found at the sarcolemma of striated and smooth muscle...
  3. Miller G, Wang E, Nassar K, Peter A, Crosbie R. Structural and functional analysis of the sarcoglycan-sarcospan subcomplex. Exp Cell Res. 2007;313:639-51 pubmed
    b>Sarcospan is a component of the dystrophin-glycoprotein complex that forms a tight subcomplex with the sarcoglycans...
  4. Scott A, Elizaga A, Morrell J, Bergen A, Penno M. Characterization of a gene coamplified with Ki-ras in Y1 murine adrenal carcinoma cells that codes for a putative membrane protein. Genomics. 1994;20:227-30 pubmed
    ..The gene, designated KRAG (Ki-ras-associated gene) has a CG-rich first exon and promoter region and a long 3' untranslated region and encodes ..
  5. Crosbie R, Lebakken C, Holt K, Venzke D, Straub V, Lee J, et al. Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex. J Cell Biol. 1999;145:153-65 pubmed
    ..We recently identified a unique tetraspan-like dystrophin-associated protein, which we have named sarcospan (SPN) for its multiple sarcolemma spanning domains (Crosbie, R.H., J. Heighway, D.P. Venzke, J.C. Lee, and K.P...
  6. Gibbs E, Marshall J, Ma E, Nguyen T, Hong G, Lam J, et al. High levels of sarcospan are well tolerated and act as a sarcolemmal stabilizer to address skeletal muscle and pulmonary dysfunction in DMD. Hum Mol Genet. 2016;25:5395-5406 pubmed publisher
    ..Previous work from our lab has shown that a small transmembrane protein called sarcospan (SSPN) can enhance the recruitment of adhesion complex proteins to the cell surface...
  7. Marshall J, Oh J, Chou E, Lee J, Holmberg J, Burkin D, et al. Sarcospan integration into laminin-binding adhesion complexes that ameliorate muscular dystrophy requires utrophin and α7 integrin. Hum Mol Genet. 2015;24:2011-22 pubmed publisher
    ..We previously demonstrated that overexpression of sarcospan, a dystrophin- and utrophin-binding protein, ameliorates mdx muscular dystrophy...
  8. Peter A, Miller G, Capote J, DiFranco M, Solares Pérez A, Wang E, et al. Nanospan, an alternatively spliced isoform of sarcospan, localizes to the sarcoplasmic reticulum in skeletal muscle and is absent in limb girdle muscular dystrophy 2F. Skelet Muscle. 2017;7:11 pubmed publisher
    b>Sarcospan (SSPN) is a transmembrane protein that interacts with the sarcoglycans (SGs) to form a tight subcomplex within the dystrophin-glycoprotein complex that spans the sarcolemma and interacts with laminin in the extracellular matrix...
  9. George D, Scott A, de Martinville B, Francke U. Amplified DNA in Y1 mouse adrenal tumor cells: isolation of cDNAs complementary to an amplified c-Ki-ras gene and localization of homologous sequences to mouse chromosome 6. Nucleic Acids Res. 1984;12:2731-43 pubmed

More Information

Publications18

  1. Parvatiyar M, Marshall J, Nguyen R, Jordan M, Richardson V, Roos K, et al. Sarcospan Regulates Cardiac Isoproterenol Response and Prevents Duchenne Muscular Dystrophy-Associated Cardiomyopathy. J Am Heart Assoc. 2015;4: pubmed publisher
    ..We have previously demonstrated that a dystrophin-associated protein, sarcospan (SSPN), ameliorated Duchenne muscular dystrophy skeletal muscle degeneration by activating compensatory pathways ..
  2. Marshall J, Chou E, Oh J, Kwok A, Burkin D, Crosbie Watson R. Dystrophin and utrophin expression require sarcospan: loss of ?7 integrin exacerbates a newly discovered muscle phenotype in sarcospan-null mice. Hum Mol Genet. 2012;21:4378-93 pubmed
    b>Sarcospan (SSPN) is a core component of the major adhesion complexes in skeletal muscle, the dystrophin- and utrophin (Utr)-glycoprotein complexes (DGC and UGC)...
  3. Marshall J, Holmberg J, Chou E, Ocampo A, Oh J, Lee J, et al. Sarcospan-dependent Akt activation is required for utrophin expression and muscle regeneration. J Cell Biol. 2012;197:1009-27 pubmed publisher
    ..We show that Akt signaling and utrophin levels were diminished in sarcospan (SSPN)-deficient muscle...
  4. Zhou D, Wang J, Zapala M, Xue J, Schork N, Haddad G. Gene expression in mouse brain following chronic hypoxia: role of sarcospan in glial cell death. Physiol Genomics. 2008;32:370-9 pubmed
    ..in the hippocampal area, and the majority of the changes in this region were upregulations; 3) two genes (Sspn and Ttc27) were downregulated in both brain regions and following both treatments; and 4) RNA interference-mediated ..
  5. Vigano M, Lamartine J, Testoni B, Merico D, Alotto D, Castagnoli C, et al. New p63 targets in keratinocytes identified by a genome-wide approach. EMBO J. 2006;25:5105-16 pubmed
    ..We discovered several new specific targets whose functional categorization links p63 to cell growth and differentiation. ..
  6. 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
    ..sarcoglycan-deficient (Sgca null, Sgcb null, Sgcg null, Sgcd null), dysferlin-deficient (Dysf null, SJL(Dysf)), sarcospan-deficient (Sspn null), and wild-type (C57Bl/6, C57Bl/10) mice...
  7. Hagiwara Y, Sasaoka T, Araishi K, Imamura M, Yorifuji H, Nonaka I, et al. Caveolin-3 deficiency causes muscle degeneration in mice. Hum Mol Genet. 2000;9:3047-54 pubmed
    ..No apparent muscle degeneration was observed in heterozygous mutant mice, indicating that pathological changes caused by caveolin-3 gene disruption were inherited through the recessive form of genetic transmission. ..
  8. 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. ..
  9. Manenti G, Stafford A, De Gregorio L, Gariboldi M, Falvella F, Avner P, et al. Linkage disequilibrium and physical mapping of Pas1 in mice. Genome Res. 1999;9:639-46 pubmed
    ..We showed the feasibility of LD in common inbred strains for the fine mapping of disease loci, and provided the biological basis and the reagents for the cloning of the Pas1 gene. ..