Recombinant Human Gamma-sarcoglycan (SGCG), partial

中文名稱：

人SGCG重組蛋白
貨號：

CSB-YP618771HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人SGCG重組蛋白
貨號：

CSB-EP618771HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人SGCG重組蛋白
貨號：

CSB-EP618771HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

人SGCG重組蛋白
貨號：

CSB-BP618771HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人SGCG重組蛋白
貨號：

CSB-MP618771HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

SGCG
Uniprot No.：

Q13326
別名：

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
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 668
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

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 showed that fifteen families were shown to carry SGCG variants in patient with early onset severe muscular dystrophy. PMID: 27759885
2. FADH2-dependent monooxygenase (SgcE6 and SgcC) that catalyzes the hydroxylation of a PCP-tethered substrate PMID: 27560143
3. These results position archvillin as a mechanically sensitive component of the dystrophin complex and demonstrate that signaling defects caused by loss of gamma-SG occur both at the sarcolemma and in the nucleus. PMID: 25605665
4. A report of two siblings with severe childhood onset limb-girdle muscular dystrophy type 2C supports the theory that the mutation G787A in the SGCG gene is a founder mutation. PMID: 24534832
5. Molecular epidemiologic methods were used to calculate the frequency of heterozygotes for this SGCG mutation in Moroccan newborns and to estimate the prevalence of LGMD2C in the Moroccan population. PMID: 24552312
6. Data suggest that an SNP in an intron of SGCG (rs9552911) is associated with type 2 diabetes [Genome-Wide Association Study in Sikh populations in India & Meta-Analysis] PMID: 23300278
7. The C allele of the c.-94C>G polymorphism in delta-sarcoglycan is a risk factor for HCM, which is increased by the Amerindian component and can play an important role in the etiology and progression of disease in Mexican patients PMID: 22524166
8. four Greek Gypsy patients with limb girdle muscular dystrophy type 2C carried the same homozygous C283Y mutation in the gamma-sarcoglycan gene PMID: 20345928
9. The relative incidence of LGMD2C among Japanese Duchenne muscular dystrophy-like patients can be calculated as 1 in 161 patients suspected to have Duchenne muscular dystrophy. PMID: 20350330
10. Clinical, histologic, and immunohistochemical characteristics of three children with limb-girdle muscular dystrophy type 2C. Two novel mutations in the gamma-sarcoglycan gene were present. We found phenotypic differences in two brothers. PMID: 15087111
11. two unrelated patients of Puerto Rican descent who have identical previously undescribed homozygous E263K (G787A) missense mutations on exon 8, and a white North American child with del521T on one allele and a deletion of exon 6 on the other allele. PMID: 16832103
12. The limb-girdle muscular dystrophy patients with gamma-sarcoglycan deficient LGMD2C do not enable an accurate prediction of the genotype. PMID: 18996010
13. This study, the first mutational analysis of Indian patients with sarcoglycanopathies suggests gamma SG mutations were the most common and the most prevalent mutation in the gamma SG gene was 525del.T. PMID: 19770540
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相關疾?。?/div>
Limb-girdle muscular dystrophy 2C (LGMD2C)
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：

Expressed in skeletal and heart muscle.
數據庫鏈接：

HGNC: 10809

OMIM: 253700

KEGG: hsa:6445

STRING: 9606.ENSP00000218867

UniGene: Hs.37167