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产品性质
| biological source【生物来源】 | rabbit |
| Quality Level【质量水平】 | 100 |
| conjugate【偶联物】 | unconjugated |
| antibody form【抗体形式】 | IgG fraction of antiserum |
| antibody product type | primary antibodies |
| clone【克隆】 | polyclonal |
| form【形式】 | buffered aqueous solution |
| mol wt【分子量】 | 46 kDa |
| species reactivity | human, horse, dog, mouse, rat |
| concentration【浓度】 | 0.5-1 mg/mL |
| technique(s) | immunoblotting: suitable immunohistochemistry: suitable |
| accession no.【登记号】 | NM_000137 |
| UniProt accession no.【UniProt登记号】 | P16930 |
| shipped in【运输】 | wet ice |
| storage temp.【储存温度】 | −20℃ |
| Gene Information | human ... FAH(2184) |
基本信息
| General description【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. |
| Immunogen【免疫原】 | Synthetic peptide directed towards the C terminal region of human FAH |
| Biochem/physiol Actions【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. |
| Sequence【序列】 | Synthetic peptide located within the following region: AATICKSNFKYMYWTMLQQLTHHSVNGCNLRPGDLLASGTISGPEPENFG |
| Physical form【外形】 | Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. |
产品说明
| Disclaimer【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. |
安全信息
| Storage Class Code【储存分类代码】 | 12 - Non Combustible Liquids |
| WGK | WGK 3 |
