SGCB Antibody

說明書

中文名稱：

SGCB兔多克隆抗體
貨號：

CSB-PA021183GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q16585
基因名：

SGCB
別名：

43 kDa dystrophin associated glycoprotein antibody; 43 kDa dystrophin-associated glycoprotein antibody; 43DAG antibody; A3b antibody; Beta SG antibody; Beta-sarcoglycan antibody; Beta-SG antibody; dystrophin-associated glycoprotein, 43-kD antibody; LGMD2E antibody; Limb girdle muscular dystrophy 2E (non linked families) antibody; Sarcoglycan beta antibody; sarcoglycan, beta (43kDa dystrophin-associated glycoprotein) antibody; SGC antibody; SGCB antibody; SGCB_HUMAN antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human SGCB
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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：
1. This study demonstrated that LGMD2E is the most common type of sarcoglycanopathies in the Iranian population. PMID: 28687063
2. Clinical severity of limb-girdle muscular dystrophy type 2Emay be predicted by SGCB gene mutation and sarcoglycan protein expression. PMID: 25862795
3. Defective assembly of sarcoglycan complex in patients with beta-sarcoglycan gene mutations PMID: 12060343
4. beta-sarcoglycan and SPATA18 may have a role in limb-girdle muscular dystrophy type 2E PMID: 16088906
5. 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
6. 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
7. 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.
數據庫鏈接：

HGNC: 10806

OMIM: 600900

KEGG: hsa:6443

STRING: 9606.ENSP00000370839

UniGene: Hs.438953

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