Target:Sarcoglycan-β
Fields:Hypertrophic cardiomyopathy;Arrhythmogenic right ventricular cardiomyopathy;Dilated cardiomyopathy;Viral myocarditis
Gene Name:SGCB
Protein Name:Beta-sarcoglycan
Human Gene Id:6443
Human Swiss Prot No:Q16585
Mouse Gene Id:24051
Mouse Swiss Prot No:P82349
Immunogen:The antiserum was produced against synthesized peptide derived from human Sarcoglycan-beta. AA range:87-136
Specificity:Sarcoglycan-β Polyclonal Antibody detects endogenous levels of Sarcoglycan-β protein.
Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source:Polyclonal, Rabbit,IgG
Dilution:WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:1 mg/ml
Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)
Other Name:SGCB;Beta-sarcoglycan;Beta-SG;43 kDa dystrophin-associated glycoprotein;43DAG;A3b
Observed Band(KD):43kD
Background: 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],
Function: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
Subcellular Location:Cell membrane, sarcolemma ; Single-pass type II membrane protein . Cytoplasm, cytoskeleton .
Expression: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.