gamma Sarcoglycan Antibody (1112101) [Unconjugated] Summary
| Immunogen |
Synthetic Peptide Accession # Q13326 |
| Specificity |
Detects a synthetic peptide specific for Human SGCG around amino acid 190 in Direct ELISA. |
| Source |
N/A |
| Isotype |
IgG1 |
| Clonality |
Monoclonal |
| Host |
Mouse |
| Purity Statement |
Protein A or G purified from hybridoma culture supernatant |
| Innovator's Reward |
Test in a species/application not listed above to receive a full credit towards a future purchase. |
Applications/Dilutions
| Dilutions |
- Immunohistochemistry 3-25 ug/mL
- Simple Western 20 ug/mL
- Western Blot 2 ug/mL
|
Packaging, Storage & Formulations
| Storage |
Use a manual defrost freezer and avoid repeated freeze-thaw cycles. - 12 months from date of receipt, -20 to -70 °C as supplied.
- 1 month, 2 to 8 °C under sterile conditions after reconstitution.
- 6 months, -20 to -70 °C under sterile conditions after reconstitution.
|
| Buffer |
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. |
| Reconstitution Instructions |
Reconstitute lyophilized material at 0.2 mg/ml in sterile PBS. For liquid material, refer to CoA for concentration. |
Notes
This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.
Alternate Names for gamma Sarcoglycan Antibody (1112101) [Unconjugated]
Background
Sarcoglycan gamma (SGCG) is
a transmembrane protein and a key component of the dystrophin-associated
glycoprotein complex (DGC), with a molecular weight of approximately 35 kDa.
The sarcoglycan complex, which includes SGCG, is critical for maintaining the
structural integrity of muscle cell membranes and for linking the actin
cytoskeleton to the extracellular matrix. SGCG is predominantly expressed in
skeletal and cardiac muscle, where it plays a crucial role in stabilizing the
muscle membrane during contraction. Mutations in the SGCG gene are
associated with limb-girdle muscular dystrophy type 2C (LGMD2C), a progressive
muscular dystrophy characterized by muscle weakness and membrane instability.
Loss of SGCG function leads to disruption of the DGC, resulting in increased
susceptibility to muscle membrane damage and impaired muscle regeneration.
Recent studies suggest that SGCG may also be involved in signaling pathways
regulating muscle homeostasis and repair. Its critical role in muscle
integrity, disease pathogenesis, and signaling underscores its potential as a
therapeutic target for the treatment of muscular dystrophies.
- Hack AA, Groh ME, McNally EM. Sarcoglycans in muscular dystrophy.
Microsc Res Tech. 2000 Feb 1-15;48(3-4):167-80. doi:
10.1002/(SICI)1097-0029(20000201/15)48:3/43.0.CO;2-T.
PMID: 10679964.
- Groh S, Zong H, Goddeeris
MM, Lebakken CS, Venzke D, Pessin JE, Campbell KP. Sarcoglycan complex:
implications for metabolic defects in muscular dystrophies. J Biol Chem. 2009
Jul 17;284(29):19178-82. doi: 10.1074/jbc.C109.010728. Epub 2009 Jun 3. PMID:
19494113; PMCID: PMC2740540.
- Bushby KM. The limb-girdle muscular dystrophies-multiple genes, multiple
mechanisms. Hum Mol Genet. 1999;8(10):1875-82. doi: 10.1093/hmg/8.10.1875.
PMID: 10469840.
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are
guaranteed for 1 year from date of receipt.
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