验证数据展示
产品信息
67986-1-PBS targets FAH in WB, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse, rat, pig samples.
经测试应用 | WB, IF/ICC, Indirect ELISA Application Description |
经测试反应性 | human, mouse, rat, pig |
免疫原 | FAH fusion protein Ag6704 种属同源性预测 |
宿主/亚型 | Mouse / IgG1 |
抗体类别 | Monoclonal |
产品类型 | Antibody |
全称 | fumarylacetoacetate hydrolase (fumarylacetoacetase) |
别名 | Fumarylacetoacetate hydrolase, FAA, EC:3.7.1.2, Beta-diketonase, 2F8E11 |
计算分子量 | 46 kDa |
观测分子量 | 40-45 kDa |
GenBank蛋白编号 | BC002527 |
基因名称 | FAH |
Gene ID (NCBI) | 2184 |
RRID | AB_2918735 |
偶联类型 | Unconjugated |
形式 | Liquid |
纯化方式 | Protein A purification |
UNIPROT ID | P16930 |
储存缓冲液 | PBS only , pH 7.3 |
储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
FAH is also named as FAA(fumarylacetoacetase), the last enzyme in tyrosine degradation, functionally important in the liver and kidney(PMID:8473520). FAH is a metabolic enzyme catalyzing the last step of tyrosine and phenylalanine catabolism: the hydrolysis of fumarylacetoacetate into acetoacetate and fumarate. In humans, deficiency of this activity is associated with the metabolic disease hereditary tyrosinaemia type I, which is also known as hepatorenal tyrosinaemia(PMID:17768357).