Browse our Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Proteins (ECHS1)

Full name:: Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Proteins (ECHS1)

On www.antibodies-online.com are 13 Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial (ECHS1) Proteins from 0 different suppliers available. A total of 98 Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial products are currently listed.

Synonyms:: C80529, cOR6not, fj55e05, SCEH, si:zc217g15.1, wu:fj55e05

list all proteins	Gene Name	GeneID	UniProt
Human ECHS1	ECHS1	1892	P30084
Rat ECHS1	ECHS1	140547	P14604
Mouse ECHS1	ECHS1	93747	Q8BH95
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Relevant Pathways for Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Proteins

Monocarboxylic Acid Catabolic Process

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More Proteins for Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Interaction Partners

Human Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial (ECHS1) interaction partners

Due to enoyl coenzyme A hydratase, short chain, 1, mitochondria (ECHS1) deficiency.
exceeding nutrients suppress Enoyl-CoA hydratase-1 (ECHS1) activity by inducing its acetylation resulting in accumulation of fatty acids and branched-chain amino acids and oncogenic mTOR (show FRAP1 Proteins) activation
ECHS1 mutations phenotype might be milder than reported earlier, compatible with prolonged survival, and also includes isolated paroxysmal exercise-induced dystonia.
Whole exome sequencing identified heterozygous ECHS1 mutations c.836T>C (novel) and c.8C>A for short-chain enoyl-CoA hydratase (SCEH) deficiency of which 1/2 of the cases are associated with secondary lymphocyte pyruvate dehydrogenase (show PDP Proteins) complex deficiency.[review]
This is the first report of homozygosity for a truncating mutation in ECHS1, which may explain the severe phenotype. Our report highlights the need to consider SCEH deficiency in patients with lethal neonatal lactic acidosis
Human ECHS1 catalyses the hydration of five substrates via different metabolic pathways, with the highest specificity for crotonyl-CoA and the lowest specificity for tiglyl-CoA
These results suggested that ECHS1 may promote cell proliferation in hepatocellular carcinoma in an EGFR (show EGFR Proteins)-dependent manner.
In conclusion, the results of the present study suggested that ECHS1 may have an important role in colorectal cancer cell proliferation and migration
identification of four additional patients with mutations in ECHS1 highlights the importance of the valine degradation pathway in Leigh syndrome
ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome.

Mouse (Murine) Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial (ECHS1) interaction partners

potential protein targets for the loss of PostC may include F(1)-ATPase gamma, Echs1 and Hsp20 (show HSPB6 Proteins) that could regulate cellular ATP consumption/production and defense response to ischaemic stress

Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial (ECHS1) Protein Profile

Protein Summary

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.

Alternative names and synonyms associated with Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial (ECHS1)

enoyl-CoA hydratase, short chain, 1, mitochondrial L homeolog (echs1.L)
enoyl-CoA hydratase, short chain 1 (ECHS1)
enoyl CoA hydratase, short chain, 1, mitochondrial (Echs1)
enoyl CoA hydratase, short chain, 1, mitochondrial (echs1)
enoyl Coenzyme A hydratase, short chain, 1, mitochondrial (Echs1)

C80529 protein
cOR6not protein
fj55e05 protein
SCEH protein
si:zc217g15.1 protein
wu:fj55e05 protein

Protein level used designations for ECHS1

enoyl Coenzyme A hydratase, short chain, 1, mitochondrial , enoyl-CoA hydratase 1 , enoyl-CoA hydratase, mitochondrial , short-chain enoyl-CoA hydratase , SCEH , short chain enoyl-CoA hydratase , Enoyl-CoA hydratase short chain 1 mitochondrial , Enoyl-CoA hydratase, short chain 1, mitochondrial , enoyl Coenzyme A hydratase, short chain 1 , mitochondrial short-chain enoyl-coenzyme A hydratase 1

GENE ID	SPECIES
733431	Xenopus laevis
1892	Homo sapiens
480828	Canis lupus familiaris
100156927	Sus scrofa
281748	Bos taurus
140547	Rattus norvegicus
770828	Gallus gallus
368912	Danio rerio
93747	Mus musculus

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