Anti-ECHS1 antibody produced in mouse

半岛bd体育手机客户端 说明

一般描述

The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature. (provided by RefSeq)

免疫原

ECHS1 (NP_004083.2, 1 a.a. ~ 290 a.a) full-length human protein.

Sequence
MAALRVLLSCARGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ

应用

Anti-ECHS1 antibody produced in mouse is suitable for indirect immunofluorescence and western blot applications.

生化/生理作用

ECHS1 (Enoyl CoA hydratase, short chain, 1, mitochondrial) performs in the mitochondrial β-oxidation of fatty acid by catalyzing the second step of the process i.e. 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, valine. Study reports that heterozygous mutation in ECHS1 causes Leigh syndrome with hypotonia, metabolic acidosis, and developmental delay symptoms.

外形

Solution in phosphate buffered saline, pH 7.4

基本信息

NACRES

NA.41

半岛bd体育手机客户端 性质

质量水平	100
生物来源	mouse
偶联物	unconjugated
抗体形式	purified immunoglobulin
antibody product type	primary antibodies
克隆	polyclonal
形式	buffered aqueous solution
分子量	antigen ~31.4 kDa
species reactivity	human
technique(s)	indirect immunofluorescence: suitable western blot: 1 μg/mL
NCBI登记号	NM_004092.2
UniProt登记号	P30084
运输	dry ice
储存温度	?20℃
Gene Information	human ... ECHS1(1892)

安全信息

储存分类代码	13 - Non Combustible Solids
WGK	WGK 1
闪点(F)	Not applicable
闪点(C)	Not applicable