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Anti-FAH

品牌
Sigma-Aldrich
货号
SAB2108553
规格纯度
IgG fraction of antiserum
参考价格
5306.13 *本价格含增值税费
促销
数量
-+
产品介绍:

产品说明

一般描述

The FAH gene is mapped to human chromosome 15q25.1. The encoded protein consists of 419 amino acids and 14 coding exons.

免疫原

Synthetic peptide directed towards the C terminal region of human FAH

生化/生理作用

FAH is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia.This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT).
FAH (fumarylacetoacetate hydrolase) catalyzes the last step in tyrosine catabolic pathway. FAH deficiency leads to hereditary tyrosinemia type 1. FAH mutations eventually lead to Renal Fanconi Syndrome, due to severe liver cirrhosis and renal tubular acidosis caused by accumulation of toxic metabolites such as fumarylacetoacetate.

序列

Synthetic peptide located within the following region: AATICKSNFKYMYWTMLQQLTHHSVNGCNLRPGDLLASGTISGPEPENFG

外形

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

产品性质

生物来源rabbit
质量水平100
偶联物unconjugated
抗体形式IgG fraction of antiserum
antibody product typeprimary antibodies
克隆polyclonal
形式buffered aqueous solution
分子量 46 kDa
species reactivityhuman, horse, dog, mouse, rat
浓度0.5-1 mg/mL
technique(s)immunoblotting: suitable
immunohistochemistry: suitable
登记号NM_000137
UniProt登记号P16930
运输wet ice
储存温度−20℃
Gene Informationhuman ... FAH(2184)

安全信息

储存分类代码12 - Non Combustible Liquids
WGKWGK 3
闪点(F)Not applicable
闪点(C)Not applicable

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