SAB1405740
Anti-ECHS1 antibody produced in mouse
purified immunoglobulin, buffered aqueous solution
别名:
SCEH
登录 查看组织和合同定价。
选择规格
变更视图
关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IF, WB
Citations:
4
biological source
mouse
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen ~31.4 kDa
species reactivity
human
technique(s)
indirect immunofluorescence: suitable, western blot: 1 μg/mL
NCBI accession no.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... ECHS1(1892)
General description
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)
Immunogen
ECHS1 (NP_004083.2, 1 a.a. ~ 290 a.a) full-length human protein.
Sequence
MAALRVLLSCARGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
Sequence
MAALRVLLSCARGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
Application
Anti-ECHS1 antibody produced in mouse is suitable for indirect immunofluorescence and western blot applications.
Biochem/physiol Actions
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.
Physical form
Solution in phosphate buffered saline, pH 7.4
Still not finding the right product?
试用我们的 产品选型工具 工具缩小选择范围
存储类别
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable