GALE Antibody, Biotin conjugated

說明書

中文名稱：

GALE兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA613499HD01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) GALE Polyclonal antibody
Uniprot No.：

Q14376
基因名：

GALE
別名：

FLJ95174 antibody; FLJ97302 antibody; Galactose 4 epimerase UDP antibody; Galactowaldenase antibody; galE antibody; GALE_HUMAN antibody; OTTHUMP00000002991 antibody; OTTHUMP00000002994 antibody; OTTHUMP00000037931 antibody; OTTHUMP00000044857 antibody; SDR1E1 antibody; short chain dehydrogenase/reductase family 1E member 1 antibody; UDP galactose 4 epimerase antibody; UDP galactose 4' epimerase antibody; UDP glucose 4 epimerase antibody; UDP-galactose 4-epimerase antibody; UDP-glucose 4-epimerase antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human UDP-glucose 4-epimerase protein (1-348AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

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

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA 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：

Catalyzes two distinct but analogous reactions: the reversible epimerization of UDP-glucose to UDP-galactose and the reversible epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The reaction with UDP-Gal plays a critical role in the Leloir pathway of galactose catabolism in which galactose is converted to the glycolytic intermediate glucose 6-phosphate. It contributes to the catabolism of dietary galactose and enables the endogenous biosynthesis of both UDP-Gal and UDP-GalNAc when exogenous sources are limited. Both UDP-sugar interconversions are important in the synthesis of glycoproteins and glycolipids.
基因功能參考文獻：
1. Mutation in UDP-galactose-4'-epimerase gene is associated with UDP-galactose-4'-epimerase deficiency. PMID: 26565537
2. Data show the protein structure of GALE and its substrate binding and specificity. It is mutated in type III galactosemia. [review] PMID: 26162744
3. human UDP-galactose 4'-epimerase stability is increased by variants associated with type III galactosemia but decreased by substrate and cofactor binding PMID: 25150110
4. These data indicated a critical role of GALE in maintaining cartilage homeostasis, and suggested that GALE inhibition might contribute to OA progress. PMID: 25201731
5. GALE variants can be arranged into three groups depending on the severity of enzyme impairment. PMID: 23644136
6. P.K161N-hGALE causes its effects by abolishing an important interaction between the protein and the cofactor. PMID: 22613355
7. study of hGALE crystal structure and demonstration that residue 307 acts as a gatekeeper mediating substrate access to the hGALE active site PMID: 15175331
8. Resulst describe the relationship among UDP-galactose 4'-epimerase activity, substrate specificity, metabolic balance, and galactose sensitivity in mammalian cells. PMID: 15701638
9. Data suggest that reduced catalytic efficiency and increased proteolytic susceptibility of UDP-galactose 4-epimerase are causative factors in type III galactosemia. PMID: 16302980
10. Subtle biochemical and metabolic abnormalities detected in patients expressing these GALE alleles likely reflect, at least in part, the reduced enzymatic activity of the encoded GALE proteins. PMID: 18188677
11. Our observations show that altered protein stability is due to misfolding and that loss or reduction of enzyme activity is responsible for the molecular defects underlying GALE-deficiency galactosemia. PMID: 19250319
12. Disease-causing mutations result in a variety of changes to the steady-state parameters. Mostly these are changes in turnover number, kcat. The ability to dimerize is not affected, but some mutants have increased sensitivity to protease digestion. PMID: 16302980
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相關疾病：

Epimerase-deficiency galactosemia (EDG)
蛋白家族：

NAD(P)-dependent epimerase/dehydratase family
數據庫鏈接：

HGNC: 4116

OMIM: 230350

KEGG: hsa:2582

STRING: 9606.ENSP00000363621

UniGene: Hs.632380

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