Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Proteine (ECHS1)

Bezeichnung:
Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Proteine (ECHS1)
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Synonyme:
C80529, cOR6not, fj55e05, SCEH, si:zc217g15.1, wu:fj55e05
alle Proteine anzeigen Gen GeneID UniProt
ECHS1 1892 P30084
ECHS1 140547 P14604
ECHS1 93747 Q8BH95

Weitere Synonyme anzeigen

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Am meisten referenzierte Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Proteine

  1. Human ECHS1 Protein expressed in Escherichia coli (E. coli) - ABIN667205 : Kanazawa, Ohtake, Abe, Yamamoto, Satoh, Takayanagi, Niimi, Mori, Hashimoto: Molecular cloning and sequence analysis of the cDNA for human mitochondrial short-chain enoyl-CoA hydratase. in Enzyme & protein 1994 (PubMed)
    Zeige alle 2 Referenzen für 667205

Weitere Proteine zu Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial Interaktionspartnern

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

  1. 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 (zeige PDP Proteine) complex deficiency.[review]

  2. 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

  3. 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

  4. These results suggested that ECHS1 may promote cell proliferation in hepatocellular carcinoma in an EGFR (zeige EGFR Proteine)-dependent manner.

  5. In conclusion, the results of the present study suggested that ECHS1 may have an important role in colorectal cancer cell proliferation and migration

  6. identification of four additional patients with mutations in ECHS1 highlights the importance of the valine degradation pathway in Leigh syndrome

  7. ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome.

  8. ECHS1 may play important roles in gastric cancer cell proliferation and migration through PKB- and GSK3b-related signaling pathways.

  9. The study demonstrated that ECHS1 mutations result in ECHS1 deficiency and are another cause of Leigh disease in two siblings.

  10. ECHS1 knockdown reduced cell viability and enhanced cisplatin-induced apoptosis in hepatocellular carcinoma cells.

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

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

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

Protein Überblick

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 (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)
  • enoyl Coenzyme A hydratase, short chain, 1, mitochondrial (Echs1)
  • C80529 Protein
  • cOR6not Protein
  • fj55e05 Protein
  • SCEH Protein
  • si:zc217g15.1 Protein
  • wu:fj55e05 Protein

Bezeichner auf Proteinebene für 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 SPEZIES
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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