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Sarcoglycan information


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 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.

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.

  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.

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Sarcoglycan

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The sarcoglycans are a family of transmembrane proteins (α, β, γ, δ or ε) involved in the protein complex responsible for connecting the muscle fibre cytoskeleton...

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Sarcoglycanopathy

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a collection of diseases resulting from mutations in any of the five sarcoglycan genes: α, β, γ, δ or ε. The five sarcoglycanopathies are: α-sarcoglycanopathy...

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SGCB

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Beta-sarcoglycan is a protein that in humans is encoded by the SGCB gene. The dystrophin-glycoprotein complex (DGC) is a multisubunit protein complex...

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Myoclonic dystonia

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Myoclonus dystonia is caused by loss-of-function-mutations in the epsilon sarcoglycan gene (SGCE). The disease is dominantly inherited, however SGCE is an...

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SGCG

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Gamma-sarcoglycan is a protein that in humans is encoded by the SGCG gene. The α to δ-sarcoglycans are expressed predominantly (β) or exclusively (α, γ...

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SGCZ

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Sarcoglycan zeta also known as SGCZ is a protein which in humans is encoded by the SGCZ gene. The zeta-sarcoglycan gene measures over 465 kb and localizes...

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List of neuromuscular disorders

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dysferlin-related LGMD R3 α-sarcoglycan-related LGMD R4 β-sarcoglycan-related LGMD R5 γ-sarcoglycan-related LGMD R6 δ-sarcoglycan-related LGMD R7 telethonin-related...

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SGCE

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Epsilon-sarcoglycan is a protein that in humans is encoded by the SGCE gene. The SGCE gene encodes the epsilon member of the sarcoglycan family, transmembrane...

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Dystrophin

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(DAPC). Many muscle proteins, such as α-dystrobrevin, syncoilin, synemin, sarcoglycan, dystroglycan, and sarcospan, colocalize with dystrophin at the costamere...

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SGCA

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Alpha-sarcoglycan is a protein that in humans is encoded by the SGCA gene. The dystrophin-glycoprotein complex (DGC) comprises a group of proteins that...

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Kifunensine

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have been identified, which result from defects in the γ-, α-, β- and δ-sarcoglycan genes. There are fewer than 1,500 patients with sarcoglycanopathy in...

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Mitochondrial myopathy

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dysferlin-related (LGMD 2B), LGMDR3 α-sarcoglycan-related (LGMD 2D), LGMDR5 γ-sarcoglycan-related (LGMD 2C), and LGMDR6 δ-sarcoglycan-related (LGMD 2F). As well...

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C10orf35

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receptor 3C, ionotropic LPAR1 Lysophosphatidic acid receptor 1 SGCD Sarcoglycan, delta (35kDa dystrophin-associated glycoprotein) ZACN Zinc activated...

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Mesoangioblast

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(DMD) and limb-girdle muscular dystrophy (LGMD). Experiments in alpha-sarcoglycan (α-SG) deficient dystrophic mice have shown that mesoangioblast transplantation...

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SGCD

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SGCD may refer to: Delta-sarcoglycan, a protein 2-Amino-4-deoxychorismate synthase, an enzyme This disambiguation page lists articles associated with...

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Integrin beta 1

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Bruschetta D, Bramanti P, Pisani A, Favaloro A (Dec 2004). "Evaluation of sarcoglycans, vinculin-talin-integrin system and filamin2 in alpha- and gamma-sarcoglycanopathy:...

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Michel Fardeau

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autosomal recessive myopathies in children with adhaline deficiency (gamma - sarcoglycan), congenital muscular dystrophies of toddlers with merosin deficiency...

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Costamere

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integral and peripheral membrane proteins such as dystroglycans and sarcoglycans, which are thought to be responsible for linking the internal cytoskeletal...

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Dystrobrevin

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α-dystrobrevin associated with dystrophin at its coil-coil region and with a sarcoglycan-protein complex at the amino-terminal. It has been proposed to function...

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Chris Ponting

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Ponting, C. P. (2002). "Cadherin-like domains in α-dystroglycan, α/ε-sarcoglycan and yeast and bacterial proteins". Current Biology. 12 (6): R197–R199...

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Integrin alpha 7

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integrin and gamma-sarcoglycan have overlapping functions in skeletal muscle. In support of this, a double knockout of gamma-sarcoglycan and alpha-7 integrin...

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Dystrobrevin alpha

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integral and peripheral membrane proteins, including dystroglycans, sarcoglycans, syntrophins and alpha- and beta-dystrobrevin. The DPC localizes to the...

Word Count : 1107

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