产品名称
Sarcoglycan-β Rabbit Polyclonal Antibody
别名
SGCB; Beta-sarcoglycan; Beta-SG; 43 kDa dystrophin-associated glycoprotein; 43DAG; A3b
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=6443
Human Swissprot No.
Q16585
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q16585/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=24051
Mouse Swissprot No.
P82349
Mouse Swissprot Link
http://www.uniprot.org/uniprot/P82349
免疫原
The antiserum was produced against synthesized peptide derived from human Sarcoglycan-beta. AA range:87-136
特异性
Sarcoglycan-β Polyclonal Antibody detects endogenous levels of Sarcoglycan-β protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a member of the sarcoglycan family. Sarcoglycans are transmembrane components in the dystrophin-glycoprotein complex which help stabilize the muscle fiber membranes and link the muscle cytoskeleton to the extracellular matrix. Mutations in this gene have been associated with limb-girdle muscular dystrophy.[provided by RefSeq, Oct 2008],
组织表达
Highest expression in heart and skeletal muscle. Low expression in brain, kidney, placenta, pancreas and lung. High expression in fetal brain. Also found in fetal lung, kidney and liver.
细胞定位
Cell membrane, sarcolemma ; Single-pass type II membrane protein . Cytoplasm, cytoskeleton .
信号通路
Hypertrophic cardiomyopathy (HCM);Arrhythmogenic right ventricular cardiomyopathy (ARVC);Dilated cardiomyopathy;Viral myocarditis;
功能
disease:Defects in SGCB are the cause of limb-girdle muscular dystrophy type 2E (LGMD2E) [MIM:604286]. LGMD2E is an autosomal recessive disorder.,function:Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.,online information:SGCB mutations in LGMD2E,PTM:Disulfide bonds are present.,similarity:Belongs to the sarcoglycan beta/delta/gamma/zeta family.,subunit:Cross-link to form 2 major subcomplexes: one consisting of SGCB, SGCD and SGCG and the other consisting of SGCB and SGCD. The association between SGCB and SGCG is particularly strong while SGCA is loosely associated with the other sarcoglycans.,tissue specificity:Highest expression in heart and skeletal muscle. Low expression in brain, kidney, placenta, pancreas and lung. High expression in fetal brain. Also found in fetal lung, kidney and liver.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
