产品说明
一般描述
ECHS1 (Enoyl CoA hydratase, short chain, 1, mitochondrial) is structurally located in chromosome 10q26.2-q26.3 region consisting of eight exons, with exons I and VIII with the 5′- and 3′-untranslated regions. It has a GC-rich 5′-flanking region with multiple copies of the SP1 binding motive. Its expressions have been found in the human liver, fibroblast and muscle.
免疫原
Synthetic peptide directed towards the C terminal region of human ECHS1
应用
Anti-ECHS1 (AB2) antibody produced in rabbit is suitable for western blotting at a concentration of 1.25μg/ml.
生化/生理作用
Enoyl CoA hydratase, short chain, 1 (ECHS1) is a mitochondrial enzyme that functions in the fatty acid β-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) to L-3-hydroxyacyl-CoAs. It also participates in different metabolic pathways involving fatty acids and amino acids. Study reports that heterozygous mutation in ECHS1 causes Leigh syndrome with hypotonia, metabolic acidosis, and developmental delay symptoms.
序列
Synthetic peptide located within the following region: KESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
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.
基本信息
NACRES | NA.41 |
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 type | primary antibodies |
克隆 | polyclonal |
形式 | buffered aqueous solution |
分子量 | 28 kDa |
species reactivity | human, rabbit |
浓度 | 0.5 mg - 1 mg/mL |
technique(s) | western blot: suitable |
NCBI登记号 | NP_004083 |
UniProt登记号 | P30084 |
运输 | wet ice |
储存温度 | −20℃ |
Gene Information | human ... ECHS1(1892) |
安全信息
储存分类代码 | 12 - Non Combustible Liquids |
WGK | WGK 3 |
闪点(F) | Not applicable |
闪点(C) | Not applicable |
Sigma-Aldrich