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Sarcoglycan

From Wikipedia, the free encyclopedia
Protein family
Sarcoglycan beta/gamma/delta
Identifiers
SymbolSarcoglycan_1
PfamPF04790
InterProIPR006875
Membranome117
Available protein structures:
PDB  IPR006875PF04790 (ECOD;PDBsum)  
AlphaFold
Protein family
Sarcoglycan alpha/epsilon
Identifiers
SymbolSarcoglycan_2
PfamPF05510
InterProIPR008908
Available protein structures:
PDB  IPR008908PF05510 (ECOD;PDBsum)  
AlphaFold

Thesarcoglycans are a family oftransmembrane proteins[1] (α, β, γ, δ or ε) involved in theprotein complex responsible for connecting the muscle fibrecytoskeleton to theextracellular matrix, preventing damage to the muscle fibresarcolemma through shearing forces.

Thedystrophin glycoprotein complex (DGC) is a membrane-spanning complex that links the interior cytoskeleton to the extracellular matrix in muscle. Thesarcoglycan 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 calledsarcoglycanopathies. Mutations in the α, β, γ or δ genes (not ε) encoding these proteins can lead to the associatedlimb-girdle muscular dystrophy.

Genes

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References

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  1. ^Sarcoglycans at the U.S. National Library of MedicineMedical Subject Headings (MeSH)
  2. ^"SGCZ sarcoglycan zeta [Homo sapiens (human)] - Gene - NCBI".www.ncbi.nlm.nih.gov. Retrieved1 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.
Smooth
muscle
Striated
muscle
Skeletal
muscle
Costamere/
DAPC
Membrane/
extracellular
DAP:
Intracellular
related:
Sarcomere/
(a, i, and h bands;
z and m lines)
Connective tissue
General
Cardiac
muscle
Both
Fiber
Cells
Other
Other/
ungrouped
This article incorporates text from the public domainPfam andInterPro:IPR006875


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