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Sarcoglycan

The sarcoglycans are a family of transmembrane proteins[1] (α, β, γ, δ or ε) involved in the protein complex responsible for connecting the muscle fibre cytoskeleton to the extracellular matrix, preventing damage to the muscle fibre sarcolemma through shearing forces.

Sarcoglycan beta/gamma/delta
Identifiers
SymbolSarcoglycan_1
PfamPF04790
InterProIPR006875
Membranome117
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary
Sarcoglycan alpha/epsilon
Identifiers
SymbolSarcoglycan_2
PfamPF05510
InterProIPR008908
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary

The dystrophin glycoprotein complex (DGC) is a membrane-spanning complex that links the interior cytoskeleton to the extracellular matrix in muscle. The sarcoglycan complex is a subcomplex within the DGC and is composed of six muscle-specific, transmembrane proteins (alpha-, beta-, gamma-, delta-, epsilon-,and zeta-sarcoglycan).[2] The sarcoglycans are asparagine-linked glycosylated proteins with single transmembrane domains.[3][4]

The disorders caused by the mutations of the sarcoglycans are called sarcoglycanopathies. Mutations in the α, β, γ or δ genes (not ε) encoding these proteins can lead to the associated limb-girdle muscular dystrophy.

Genes edit

References edit

  1. ^ Sarcoglycans at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
  2. ^ "SGCZ sarcoglycan zeta [Homo sapiens (human)] - Gene - NCBI". www.ncbi.nlm.nih.gov. Retrieved 1 December 2021.
  3. ^ Chockalingam PS, Cholera R, Oak SA, Zheng Y, Jarrett HW, Thomason DB (August 2002). "Dystrophin-glycoprotein complex and Ras and Rho GTPase signaling are altered in muscle atrophy". American Journal of Physiology. Cell Physiology. 283 (2): C500-11. doi:10.1152/ajpcell.00529.2001. PMID 12107060.
  4. ^ Wheeler MT, Zarnegar S, McNally EM (September 2002). "Zeta-sarcoglycan, a novel component of the sarcoglycan complex, is reduced in muscular dystrophy". Human Molecular Genetics. 11 (18): 2147–54. doi:10.1093/hmg/11.18.2147. PMID 12189167.
This article incorporates text from the public domain Pfam and InterPro: IPR006875

sarcoglycan, sarcoglycans, family, transmembrane, proteins, involved, protein, complex, responsible, connecting, muscle, fibre, cytoskeleton, extracellular, matrix, preventing, damage, muscle, fibre, sarcolemma, through, shearing, forces, beta, gamma, deltaide. The sarcoglycans are a family of transmembrane proteins 1 a b g d or e involved in the protein complex responsible for connecting the muscle fibre cytoskeleton to the extracellular matrix preventing damage to the muscle fibre sarcolemma through shearing forces Sarcoglycan beta gamma deltaIdentifiersSymbolSarcoglycan 1PfamPF04790InterProIPR006875Membranome117Available protein structures Pfam structures ECOD PDBRCSB PDB PDBe PDBjPDBsumstructure summary Sarcoglycan alpha epsilonIdentifiersSymbolSarcoglycan 2PfamPF05510InterProIPR008908Available protein structures Pfam structures ECOD PDBRCSB PDB PDBe PDBjPDBsumstructure summary The dystrophin glycoprotein complex DGC is a membrane spanning complex that links the interior cytoskeleton to the extracellular matrix in muscle The sarcoglycan complex is a subcomplex within the DGC and is composed of six muscle specific transmembrane proteins alpha beta gamma delta epsilon and zeta sarcoglycan 2 The sarcoglycans are asparagine linked glycosylated proteins with single transmembrane domains 3 4 The disorders caused by the mutations of the sarcoglycans are called sarcoglycanopathies Mutations in the a b g or d genes not e encoding these proteins can lead to the associated limb girdle muscular dystrophy Genes editSGCA SGCB SGCD SGCE SGCG SGCZReferences edit Sarcoglycans at the U S National Library of Medicine Medical Subject Headings MeSH SGCZ sarcoglycan zeta Homo sapiens human Gene NCBI www ncbi nlm nih gov Retrieved 1 December 2021 Chockalingam PS Cholera R Oak SA Zheng Y Jarrett HW Thomason DB August 2002 Dystrophin glycoprotein complex and Ras and Rho GTPase signaling are altered in muscle atrophy American Journal of Physiology Cell Physiology 283 2 C500 11 doi 10 1152 ajpcell 00529 2001 PMID 12107060 Wheeler MT Zarnegar S McNally EM September 2002 Zeta sarcoglycan a novel component of the sarcoglycan complex is reduced in muscular dystrophy Human Molecular Genetics 11 18 2147 54 doi 10 1093 hmg 11 18 2147 PMID 12189167 This article incorporates text from the public domain Pfam and InterPro IPR006875 nbsp This protein related article is a stub You can help Wikipedia by expanding it vte Retrieved from https en wikipedia org w index php title Sarcoglycan amp oldid 1058096488, wikipedia, wiki, book, books, library,

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