Browse our anti-Enoyl CoA Hydratase, Short Chain, 1, Mitochondrial (ECHS1) Antibodies

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

1. Due to enoyl coenzyme A hydratase, short chain, 1, mitochondria (ECHS1) deficiency.

2. 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 activation

3. ECHS1 mutations phenotype might be milder than reported earlier, compatible with prolonged survival, and also includes isolated paroxysmal exercise-induced dystonia.

4. 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 complex deficiency.[review]

5. 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

6. 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

7. These results suggested that ECHS1 may promote cell proliferation in hepatocellular carcinoma in an EGFR-dependent manner.

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

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

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

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

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

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

14. The results confirmed that small hepatitis B surface antigen (SHBs) interacted with ECHS1.

15. The coexistence of HBs and ECHS1 enhances HepG2 cell apoptosis, affects ECHS1 localization in the mitochondria and induces apoptosis by decreasing the mitochondrial membrane potential (MMP).

16. ECHS1 specifically represses STAT3 activity and negatively regulates the expression of several target genes of STAT3 through inhibiting STAT3 phosphorylation.

17. Expression of mitochondrial short chain enoyl-CoA hydratase (ECHS)was significantly down-modulated in virus infected glioblastoma cells and ECHS knockdown (siRNA) impaired virus replication and cytopathic effects.

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

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