EHHADH
In the C-terminal section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family. Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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Protein type: Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - propanoate; EC 1.1.1.35; EC 4.2.1.17; EC 5.3.3.8; Isomerase; Lipid Metabolism - fatty acid; Lyase; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Oxidoreductase; Secondary Metabolites Metabolism - limonene and pinene degradation |
Chromosomal Location of human Ortholog: 3q27.2 |
Cellular Component:
cytosol; peroxisomal matrix; peroxisome
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Molecular Function:
3-hydroxyacyl-CoA dehydratase activity; 3-hydroxyacyl-CoA dehydrogenase activity; delta(3)-delta(2)-enoyl-CoA isomerase activity; enoyl-CoA hydratase activity; enzyme binding; intramolecular oxidoreductase activity, transposing C=C bonds; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; NAD+ binding; protein binding
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Biological Process:
fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA oxidase
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Disease: Fanconi Renotubular Syndrome 3
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Reference #:
Q08426
(UniProtKB)
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Alt. Names/Synonyms: 3,2-trans-enoyl-CoA isomerase; 3-hydroxyacyl-CoA dehydrogenase; ECHD; ECHP; EHHADH; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase; Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase; enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase; enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase; FRTS3; L-3-hydroxyacyl-CoA dehydrogenase; L-bifunctional protein; L-bifunctional protein, peroxisomal; L-PBE; LBFP; LBP; MFE1; MGC120586; Multifunctional enzyme 1; PBE; PBFE; Peroxisomal bifunctional enzyme; peroxisomal enoyl-CoA hydratase
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Gene Symbols: EHHADH
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Molecular weight:
79,495 Da
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Basal Isoelectric point:
9.24
Predict pI for various phosphorylation states
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