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Product Name alpha Sarcoglycan Chinese Name α肌萎缩glycoprotein2抗体 Alias 50 DAG; 50 kDa dystrophin associated glycoprotein; 50 kDa dystrophin-associated glycoprotein; 50DAG; 50kD DAG; 59kDa; A2; adhalin; ADL; Alpha SG; Alpha-sarcoglycan; Alpha-SG; Asg; DAG2; DMDA2; Dystroglycan 2; Dystroglycan-2; LGMD2D; sarcoglycan, alpha (dystrophin-associated glycoprotein); SCARMD1; Sgca; SGCA_HUMAN. Research Area Cell biology Neurobiology Signal transduction glycoprotein Immunogen Species Rabbit Clonality Polyclonal React Species Rat, (predicted: Human, Mouse, Dog, Pig, Rabbit, ) Applications WB=1:500-2000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 40kDa Cellular localization cytoplasmic The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human alpha Sarcoglycan: 51-150/387 <Extracellular> Lsotype IgG Purification affinity purified by Protein A Buffer Solution Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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.
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.
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 (By similarity).
Subcellular Location:
Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
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.
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.
Similarity:
Belongs to the sarcoglycan alpha/epsilon family.
SWISS:
Q16586
Gene ID:
6442
Database links:Entrez Gene: 6442 Human
Entrez Gene: 20391 Mouse
Omim: 600119 Human
SwissProt: Q16586 Human
SwissProt: P82350 Mouse
SwissProt: Q5SWB2 Mouse
SwissProt: Q28686 Rabbit
Unigene: 463412 Human
Unigene: 18709 Mouse
Unigene: 136653 Rat
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