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gamma Sarcoglycan Rabbit pAb
- 产品详情
- 实验流程
Application 
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q13326 |
| Other Accession | Q13326 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 32379 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human gamma Sarcoglycan |
| Epitope Specificity | 151-250/291 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the sarcoglycan beta/delta/gamma/zeta 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 (By similarity). Interacts with FLNC. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 6445 |
|---|---|
| Other Names | 35DAG; A4; DAGA4; DMDA; DMDA1; LGMD2C; LGMDR5; MAM; SCARMD2; gamma-SG; SGCG_HUMAN; SGCG; 35 kDa dystrophin-associated glycoprotein (35DAG); sarcoglycan gamma; sarcoglycan, gamma (35kDa dystrophin-associated glycoprotein); Maghrebian myopathy (autosomal recessive); 35kD dystrophin-associated glycoprotein; limb girdle muscular dystrophy 2C (Duchenne-like muscular dystrophy, autosomal recessive); gamma sarcoglycan |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | SGCG |
|---|---|
| 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. |
| Cellular Location | Cell membrane, sarcolemma; Single- pass type II membrane protein. Cytoplasm, cytoskeleton |
| Tissue Location | Expressed in skeletal and heart muscle. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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¥ 1,250.00
Cat# AP99271
