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Key Documents

WH0001892M1

Sigma-Aldrich

Monoclonal Anti-ECHS1 antibody produced in mouse

clone 1G9, ascites fluid

Synonym(s):

Anti-SCEH, Anti-enoyl Coenzyme A hydratase, short chain, 1, mitochondrial

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

Quality Level

100

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.

BC008906

UniProt accession no.

P30084

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... ECHS1(1892)

Related Categories

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

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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Storage Class Code

10 - Combustible liquids

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

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Certificates of Analysis (COA)

Lot/Batch Number
08185-1G9

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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)
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
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
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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