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Product Name Anti-gamma Sarcoglycan/Gold Chinese Name 胶体金标记的γ-肌聚糖/肌聚糖伽玛/γ-sarcoglycan抗体 Alias 35 kDa dystrophin associated glycoprotein; 35 kDa dystrophin-associated glycoprotein; 35DAG; 35kD dystrophin associated glycoprotein; 35kDa dystrophin-associated glycoprotein; A4; DAGA4; DMDA; DMDA1; Gamma SG; Gamma-sarcoglycan; Gamma-SG; LGMD2C; MAM; MGC130048; Sarcoglycan gamma; SCARMD2; SCG3; SGCG; SGCG_HUMAN; TYPE. Research Area Cell biology immunology Signal transduction Cytoskeleton Transmembrane protein Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Sheep, ) Applications IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 32kDa Form Lyophilized or Liquid Concentration 0.4mg/ml immunogen KLH conjugated synthetic peptide derived from human gamma Sarcoglycan Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. Storage Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles. Product Detail background:
This gene encodes gamma-sarcoglycan, one of several sarcolemmal transmembrane glycoproteins that interact with dystrophin. The dystrophin-glycoprotein complex (DGC) spans the sarcolemma and is comprised of dystrophin, syntrophin, alpha- and beta-dystroglycans and sarcoglycans. The DGC provides a structural link between the subsarcolemmal cytoskeleton and the extracellular matrix of muscle cells. Defects in the encoded protein can lead to early onset autosomal recessive muscular dystrophy, in particular limb-girdle muscular dystrophy, type 2C (LGMD2C). [provided by RefSeq, Oct 2008]
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). Interacts with FLNC.
Subcellular Location:
Cell membrane; sarcolemma. Cytoplasm > cytoskeleton.
Tissue Specificity:
Expressed in skeletal and heart muscle.
DISEASE:
Defects in SGCG are the cause of limb-girdle muscular dystrophy type 2C (LGMD2C) [MIM:253700]. LGMD2C is characterized by progressive muscle wasting from early childhood.
Similarity:
Belongs to the sarcoglycan beta/delta/gamma/zeta family.
Database links:Entrez Gene: 6445 Human
Omim: 608896 Human
SwissProt: Q13326 Human
Unigene: 37167 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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