IHC image of CSB-PA618082LA01HU diluted at 1:600 and staining in paraffin-embedded human melanoma performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Immunofluorescence staining of Hela cells with CSB-PA618082LA01HU at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
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用途:
For Research Use Only. Not for use in diagnostic or therapeutic procedures.
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.
基因功能參考文獻:
This study demonstrated that LGMD2E is the most common type of sarcoglycanopathies in the Iranian population. PMID: 28687063
Clinical severity of limb-girdle muscular dystrophy type 2Emay be predicted by SGCB gene mutation and sarcoglycan protein expression. PMID: 25862795
Defective assembly of sarcoglycan complex in patients with beta-sarcoglycan gene mutations PMID: 12060343
beta-sarcoglycan and SPATA18 may have a role in limb-girdle muscular dystrophy type 2E PMID: 16088906
While the quantity of beta-sarcoglycan was nearly normal in the limb girdle muscular dystrophy (LGMD)2E carrier, the levels of dysferlin protein were reduced to 50% of controls in the carriers of LGMD2B. PMID: 16934466
These data suggest that formation of the beta-delta-core may promote the export and deposition of sarcoglycan subcomplexes at the plasma membrane, and therefore identifies a mechanism for sarcoglycan transport. PMID: 17036316
The limb-girdle muscular dystrophy patients with beta-sarcoglycan deficient LGMD2E do not enable an accurate prediction of the genotype. PMID: 18996010
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相關疾病:
Limb-girdle muscular dystrophy 2E (LGMD2E)
亞細胞定位:
Cell membrane, sarcolemma; Single-pass type II membrane protein. Cytoplasm, cytoskeleton.
蛋白家族:
Sarcoglycan beta/delta/gamma/zeta family
組織特異性:
Highest expression in heart and skeletal muscle. Low expression in brain, kidney, placenta, pancreas and lung. High expression in fetal brain. Also found in fetal lung, kidney and liver.