ITT4215
ITT4215
- Catalog: ITT4215
- Gene/Protein: SGCA
- Product Description: Immunotag™ Sarcoglycan α Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
|
| Target Protein | Sarcoglycan α |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from human SGCA. AA range:161-210 |
| Specificity | Sarcoglycan α Polyclonal Antibody detects endogenous levels of Sarcoglycan α protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | SGCA |
| Accession No. | Q16586 P82350 |
| Alternate Names | SGCA; ADL; DAG2; Alpha-sarcoglycan; Alpha-SG; 50 kDa dystrophin-associated glycoprotein; 50DAG; Adhalin; Dystroglycan-2 |
| Description | sarcoglycan alpha(SGCA) Homo sapiens This gene encodes a component of the dystrophin-glycoprotein complex (DGC), which is critical to the stability of muscle fiber membranes and to the linking of the actin cytoskeleton to the extracellular matrix. Its expression is thought to be restricted to striated muscle. Mutations in this gene result in type 2D autosomal recessive limb-girdle muscular dystrophy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2008], |
| Cell Pathway/ Category | Hypertrophic cardiomyopathy (HCM),Arrhythmogenic right ventricular cardiomyopathy (ARVC),Dilated cardiomyopathy,Viral myocarditis, |
| Protein Expression | Heart,Heart ventricle,Pancreas,Skeletal muscle,Spleen, |
| Subcellular Localization | cytoplasm,cytoskeleton,cell-cell junction,dystrophin-associated glycoprotein complex,sarcoglycan complex,integral component of membrane,sarcolemma,membrane raft, |
| Protein Function | disease:Defects in SGCA are the cause of limb-girdle muscular dystrophy type 2D (LGMD2D) [MIM:608099]; also known as Duchenne-like muscular dystrophy autosomal recessive type 2 or severe childhood autosomal recessive muscular dystrophy (SCARMD). LGMD2D is an autosomal recessive degenerative myopathy characterized by progressive muscle wasting from early childhood with loss of independent ambulation by teenage years. Muscle biopsy shows necrosis, decreased immunostaining for alpha sarcoglycan, and adhalin deficiency. The phenotype is less severe than LGMD2C.,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:SGCA mutations in LGMD2D,similarity:Belongs to the sarcoglycan alpha/epsilon family.,subunit:Interacts with the syntrophin SNTA1. 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:Most strongly expressed in skeletal muscle. Also expressed in cardiac muscle and, at much lower levels, in lung. In the fetus, most abundant in cardiac muscle and, at lower levels, in lung. Also detected in liver and kidney. Not expressed in brain., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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