NP_004083.3
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
31,387 Da
NCBI Official Full Name
enoyl-CoA hydratase, mitochondrial
NCBI Official Synonym Full Names
enoyl-CoA hydratase, short chain, 1, mitochondrial
NCBI Official Synonym Symbols
NCBI Protein Information
enoyl-CoA hydratase, mitochondrial
UniProt Protein Name
Enoyl-CoA hydratase, mitochondrial
UniProt Synonym Protein Names
Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEH
UniProt Synonym Gene Names
UniProt Entry Name
ECHM_HUMAN
NCBI Summary for ECHS1
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, Jul 2008]
UniProt Comments for ECHS1
ECHS1: Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Belongs to the enoyl-CoA hydratase/isomerase family.
Protein type: EC 4.2.1.17; Lipid Metabolism - fatty acid; Lyase; Other Amino Acids Metabolism - beta-alanine; Lipid Metabolism - fatty acid elongation in mitochondria; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Amino Acid Metabolism - tryptophan; Secondary Metabolites Metabolism - limonene and pinene degradation; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - lysine degradation
Chromosomal Location of Human Ortholog: 10q26.2-q26.3
Cellular Component: mitochondrial matrix; mitochondrion
Molecular Function: enoyl-CoA hydratase activity; protein binding
Biological Process: cellular lipid metabolic process; fatty acid beta-oxidation
Disease: Mitochondrial Short-chain Enoyl-coa Hydratase 1 Deficiency
Research Articles on ECHS1
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Products associated with ECHS1 elisa kit
Pathways associated with ECHS1 elisa kit
Diseases associated with ECHS1 elisa kit
Organs/Tissues associated with ECHS1 elisa kit
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