Gene Ontology annotation through association of InterPro records with GO terms
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on curation of immunofluorescence data
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Automatic Gene Ontology annotation based on Rhea mapping
Electronic Gene Ontology annotations created by ARBA machine learning models
Combined Automated Annotation using Multiple IEA Methods
AIP is a mitochondrial import mediator that binds to both import receptor Tom20 and preproteins.
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ECHS1 appears in this study as a model imported mitochondrial preprotein/matrix protein; the AIP-Tom20-preprotein system mediates mitochondrial protein import.
"AIP is a mitochondrial import mediator that binds to both import receptor Tom20"
Proteomics of human umbilical vein endothelial cells applied to etoposide-induced apoptosis.
ECHS1 interacts with STAT3 and negatively regulates STAT3 signaling.
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ECHS1 was identified as a STAT3-binding protein that represses STAT3 activity by inhibiting STAT3 phosphorylation, suggesting a possible regulatory/moonlighting role.
"we identified enoyl-CoA hydratase short chain 1 (ECHS1) as a novel STAT3 binding protein"
Unbiased screen for interactors of leucine-rich repeat kinase 2 supports a common pathway for sporadic and familial Parkinson disease.
Differential protein-protein interactions of LRRK1 and LRRK2 indicate roles in distinct cellular signaling pathways.
Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion.
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Purified human ECHS1 hydrates five enoyl-CoA substrates across different metabolic pathways, with highest specificity for crotonyl-CoA and lowest for tiglyl-CoA, spanning fatty acid beta-oxidation and branched-chain amino acid catabolism.
"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."
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ECHS1 deficiency impairs both valine catabolic and beta-oxidation pathways; affected patients excrete N-acetyl-S-(2-carboxypropyl)cysteine, a methacrylyl-CoA metabolite.
"harbouring defective valine catabolic and β-oxidation pathways"
Parkinson's disease-associated LRRK2-G2019S mutant acts through regulation of SERCA activity to control ER stress in astrocytes.
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
Lysine catabolism reprograms tumour immunity through histone crotonylation.
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ECHS1 is the crotonyl-CoA hydratase; its downregulation accumulates crotonyl-CoA (a lysine-catabolism intermediate) and increases histone H4 lysine crotonylation.
"downregulation of the crotonyl-CoA hydratase enoyl-CoA hydratase short chain 1 (ECHS1), leading to accumulation of intracellular crotonyl-CoA and histone H4 lysine crotonylation"
Ectopic protein lysine methacrylation contributes to defects caused by loss of HIBCH or ECHS1.
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Loss of ECHS1 (or downstream HIBCH) elevates lysine methacrylation and causes abnormal mitochondrial morphology and respiratory defects, consistent with build-up of the valine-pathway intermediate methacrylyl-CoA that ECHS1 normally hydrates.
"Elevated lysine methacrylation (Kmea) is observed in both HIBCH- and ECHS1-deficient cells and fly tissues"
Human mitochondrial enoyl-CoA hydratase gene (ECHS1): structural organization and assignment to chromosome 10q26.2-q26.3.
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ECHS1 (EC 4.2.1.17) catalyzes the second step of mitochondrial fatty acid beta-oxidation; the gene maps to chromosome 10q26.2-q26.3.
"The second step in mitochondrial fatty acid beta-oxidation is catalyzed by short"
ECHS1 hydrates methacrylyl-CoA
Branched-chain amino acid catabolism
2-trans-Dodecenoyl-CoA+H2O =>( S)-3-Hydroxydodecanoyl-CoA
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA
Crotonoyl-CoA+H2O => (S)-3-Hydroxybutanoyl-CoA
trans-Hex-2-enoyl-CoA+H2O<=>(S)-Hydroxyhexanoyl-CoA
trans-Oct-2-enoyl-CoA+H2O => (S)-Hydroxyoctanoyl-CoA
trans-Dec-2-enoyl-CoA+H2O => (S)-Hydroxydecanoyl-CoA
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA
Beta oxidation of octanoyl-CoA to hexanoyl-CoA
Beta oxidation of hexanoyl-CoA to butanoyl-CoA
Beta oxidation of butanoyl-CoA to acetyl-CoA
ECHS1 mutants don't synthesize beta-hydroxyisobutyryl-CoA