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SAB1405740

Sigma-Aldrich

Anti-ECHS1 antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

Synonym(s):

SCEH

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50 μG
$541.00

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50 μG
$541.00

About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

$541.00

Ships within 2 weeks. (Orders outside of US and Europe, please allow an additional 1-2 weeks for delivery)
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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.

NM_004092.2

UniProt accession no.

P30084

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

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

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Storage Class

10 - Combustible liquids

wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificates of Analysis (COA)

Lot/Batch Number
10258

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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)
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
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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