NP_004083.3
[Other Products]
NCBI GenBank Nucleotide #
|
[Other Products]
UniProt Primary Accession #
|
[Other Products]
UniProt Secondary Accession #
UniProt Related Accession #
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; enoyl Coenzyme A hydratase, short chain, 1, mitochondrial; enoyl-CoA hydratase 1; short-chain enoyl-CoA hydratase
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; Secondary Metabolites Metabolism - limonene and pinene degradation; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; Lyase; Lipid Metabolism - fatty acid; Amino Acid Metabolism - lysine degradation; Lipid Metabolism - fatty acid elongation in mitochondria; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Amino Acid Metabolism - tryptophan; Carbohydrate Metabolism - propanoate
Chromosomal Location of Human Ortholog: 10q26.2-q26.3
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: protein binding; enoyl-CoA hydratase activity
Biological Process: fatty acid beta-oxidation; cellular lipid metabolic process
Disease: Mitochondrial Short-chain Enoyl-coa Hydratase 1 Deficiency
Research Articles on ECHS1
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice. It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Products associated with ECHS1 recombinant protein
Pathways associated with ECHS1 recombinant protein
Diseases associated with ECHS1 recombinant protein
Organs/Tissues associated with ECHS1 recombinant protein
|