WH0001892M1
Monoclonal Anti-ECHS1 antibody produced in mouse
clone 1G9, ascites fluid
别名:
Anti-SCEH, Anti-enoyl Coenzyme A hydratase, short chain, 1, mitochondrial
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
1G9, monoclonal
Application:
ELISA (i), IP
Citations:
4
biological source
mouse
conjugate
unconjugated
antibody form
ascites fluid
antibody product type
primary antibodies
clone
1G9, monoclonal
species reactivity
human
technique(s)
immunoprecipitation (IP): suitable, indirect ELISA: suitable
isotype
IgMκ
GenBank accession no.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... ECHS1(1892)
Immunogen
ECHS1 (AAH08906, 13 a.a. ~ 290 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
GPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
Sequence
GPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
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
Legal Information
GenBank is a registered trademark of United States Department of Health and Human Services
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存储类别
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
常规特殊物品
此项目有
M Kanazawa et al.
Enzyme & protein, 47(1), 9-13 (1993-01-01)
Short chain enoyl-CoA hydratase (SCEH) catalyzes the second step of the mitochondrial fatty acid beta-oxidation spiral. We isolated cDNA clones for human SCEH to facilitate investigation of the enzyme structure of the gene and to examine the genetic background of
U Janssen et al.
Genomics, 40(3), 470-475 (1997-03-15)
The second step in mitochondrial fatty acid beta-oxidation is catalyzed by short chain enoyl-CoA hydratase (ECHS1; EC 4.2.1.17). Inherited disorders of this pathway of energy metabolism present clinical and laboratory features resembling sudden infant death syndrome and Reye-like syndrome. To
Enzymes of fatty acid metabolism. II. Properties of crystalline crotonase.
J R STERN et al.
The Journal of biological chemistry, 218(2), 985-1002 (1956-02-01)
Chika Sakai et al.
Human mutation, 36(2), 232-239 (2014-11-14)
The human ECHS1 gene encodes the short-chain enoyl coenzyme A hydratase, the enzyme that catalyzes the second step of β-oxidation of fatty acids in the mitochondrial matrix. We report on a boy with ECHS1 deficiency who was diagnosed with Leigh
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