Description
| 抗体名: | SGCA Antibody (PACO22138) |
| 抗体コード: | PACO22138 |
| サイズ: | 100ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB |
| 推奨される希釈: | ELISA:1:2000-1:10000, WB:1:500-1:3000 |
| 反応性: | Human |
| 免疫原: | Synthesized peptide derived from internal of human SGCA. |
| 憲法: | Liquid |
| ストレージバッファ: | Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
| 精製方法: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
 | Western blot analysis of extracts from Jurkat cells, HepG2 cells and HeLa cells, using SGCA antibody. |
| バックグラウンド: | 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. |
| シノニム: | Alpha-sarcoglycan; Alpha-SG; Adhalin; 50 kDa dystrophin-associated glycoprotein; 50DAG |
| UniProt Protein Function: | SGCA: 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. Defects in SGCA are the cause of limb-girdle muscular dystrophy type 2D (LGMD2D); 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. Belongs to the sarcoglycan alpha/epsilon family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Motility/polarity/chemotaxis; Membrane protein, integral Chromosomal Location of Human Ortholog: 17q21 Cellular Component: dystrophin-associated glycoprotein complex; sarcoglycan complex Molecular Function:protein binding Biological Process: muscle contraction; muscle development Disease: Muscular Dystrophy, Limb-girdle, Type 2d |
| NCBI Summary: | 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] |
| UniProt Code: | Q16586 |
| NCBI GenInfo Identifier: | 13431858 |
| NCBI Gene ID: | 6442 |
| NCBI Accession: | Q16586.1 |
| UniProt Secondary Accession: | Q16586,Q13710, Q13712, A6NEB8, A8K3K7, |
| UniProt Related Accession: | Q16586 |
| Molecular Weight: | 29,354 Da |
| NCBI Full Name: | Alpha-sarcoglycan |
| NCBI Synonym Full Names: | sarcoglycan alpha |
| NCBI Official Symbol: | SGCA |
| NCBI Official Synonym Symbols: | ADL; DAG2; 50DAG; DMDA2; LGMD2D; SCARMD1; adhalin |
| NCBI Protein Information: | alpha-sarcoglycan |
| UniProt Protein Name: | Alpha-sarcoglycan |
| UniProt Synonym Protein Names: | 50 kDa dystrophin-associated glycoprotein; 50DAG; Adhalin; Dystroglycan-2 |
| Protein Family: | Alpha-sarcoglycan |
| UniProt Gene Name: | SGCA |
| UniProt Entry Name: | SGCA_HUMAN |
