深圳欣博盛生物科技有限公司 a1

GALE antibody
货号:GTX33212 规格:100μl 目录价:¥5000
产品详情
* 以下信息仅供参考,详情请以原厂网站为准
产品名称:
GALE antibody
别名:
UDP-galactose-4-epimerase , SDR1E1
反应种属:
Human, Mouse, Rat
宿主来源:
Rabbit
实验应用:
ICC/IF, WB
同种型:
IgG
免疫原:
Recombinant fusion protein containing a sequence corresponding to amino acids 129-348 of human GALE (NP_001121093.1).
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by affinity chromatography
偶联:
Unconjugated
产品浓度:
Batch dependent (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
38
产品形式:
Liquid
存储溶液:
PBS, 50% Glycerol, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
This gene encodes UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and cognitive disability, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form). Multiple alternatively spliced transcripts encoding the same protein have been identified. [provided by RefSeq, Jul 2008]
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