| 词条 | Sarcoglycan |
| 释义 |
| Symbol = Sarcoglycan_1 | Name = Sarcoglycan beta/gamma/delta | image = | width = | caption = | Pfam = PF04790 | InterPro = IPR006875 | SMART = | PROSITE = | SCOP = | TCDB = | OPM family = | OPM protein = | PDB = | Membranome superfamily = 117 }}{{Pfam_box | Symbol = Sarcoglycan_2 | Name = Sarcoglycan alpha/epsilon | image = | width = | caption = | Pfam = PF05510 | InterPro = IPR008908 | SMART = | PROSITE = | SCOP = | TCDB = | OPM family = | OPM protein = | PDB = }} 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 several muscle-specific, transmembrane proteins (alpha-, beta-, gamma-, delta- and zeta-sarcoglycan). The sarcoglycans are asparagine-linked glycosylated proteins with single transmembrane domains.[2][3] 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
References1. ^{{MeshName|Sarcoglycans}} {{Muscle tissue}}{{InterPro content|IPR006875}}{{protein-stub}}2. ^{{cite journal | vauthors = Chockalingam PS, Cholera R, Oak SA, Zheng Y, Jarrett HW, Thomason DB | title = Dystrophin-glycoprotein complex and Ras and Rho GTPase signaling are altered in muscle atrophy | journal = American Journal of Physiology. Cell Physiology | volume = 283 | issue = 2 | pages = C500-11 | date = August 2002 | pmid = 12107060 | doi = 10.1152/ajpcell.00529.2001 }} 3. ^{{cite journal | vauthors = Wheeler MT, Zarnegar S, McNally EM | title = Zeta-sarcoglycan, a novel component of the sarcoglycan complex, is reduced in muscular dystrophy | journal = Human Molecular Genetics | volume = 11 | issue = 18 | pages = 2147–54 | date = September 2002 | pmid = 12189167 | doi = 10.1093/hmg/11.18.2147 }} 1 : Protein families |
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