| 621441 | Ehhadh | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity; delta(3)-delta(2)-enoyl-CoA isomerase activity; enoyl-CoA hydratase activity; INVOLVED IN fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA oxidase (ortholog); fatty acid biosynthetic process (ort holog); PARTICIPATES IN valproic acid pharmacokinetics pathway; beta-alanine metabolic pathway; butanoate metabolic pathway; ASSOCIATED WITH cholestasis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol; peroxisome; peroxisomal matrix (ortholog); INTERACTS WITH (+)-schisandrin B; 1-benzylpiperazine; 1-naphthyl isothiocyanate | 11 | 92746409 | 92779647 | Rat | 389 | symbol , PhenoGen | gene, protein-coding, PROVISIONAL [RefSeq] |
| 731537 | Ehhadh | enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase | Enables 3-hydroxyacyl-CoA dehydratase activity and 3-hydroxyacyl-CoA dehydrogenase activity. Involved in fatty acid derivative biosynthetic process and fatty acid metabolic process. Acts upstream of or within fatty acid beta-oxidation. Located in mitochondrion. Is active in peroxisomal matrix. Is ex pressed in several structures, including alimentary system; brown fat; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Fanconi renotubular syndrome 3. Orthologous to human EHHADH (enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase). [provided by Alliance of Genome Resources, Apr 2025] | 16 | 21580035 | 21606589 | Mouse | 416 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 1349142 | EHHADH | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defect s in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009] | 3 | 185190624 | 185254049 | Human | 437 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 11748471 | EHHADH | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); intramolecular oxidoreductase activity, transposing C=C bonds (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholest asis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog) | 3 | 182228520 | 182319339 | Bonobo | 52 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12053832 | EHHADH | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); intramolecular oxidoreductase activity, transposing C=C bonds (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholest asis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog); INTERACTS WITH phenobarbital | 34 | 18021047 | 18080993 | Dog | 53 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 13878399 | EHHADH | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); intramolecular oxidoreductase activity, transposing C=C bonds (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholest asis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog); INTERACTS WITH deoxynivalenol | | | | Pig | 53 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155236311 | ehhadh | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 18933541 | Ehhadh | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); 3-hydroxyacyl-CoA dehydratase activity (ortholog); enzyme binding (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); fatty acid beta-oxidation using acyl-CoA oxidase (ortholog); fatty acid bio synthetic process (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholestasis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog); peroxisomal matrix (ortholog) | | | | Naked Mole-Rat | 49 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18633882 | EHHADH | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); intramolecular oxidoreductase activity, transposing C=C bonds (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholest asis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog) | | | | Green Monkey | 52 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 626098716 | Ehhadh | enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); 3-hydroxyacyl-CoA dehydratase activity (ortholog); delta(3)-delta(2)-enoyl-CoA isomerase activity (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); fatty acid beta-oxidation using acyl-CoA ox idase (ortholog); fatty acid biosynthetic process (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholestasis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog); peroxisomal matrix (ortholog); peroxisome (ortholog) | | | | Black Rat | 40 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155236310 | ehhadh.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 6767176 | EHHADH-AS1 | EHHADH antisense RNA 1 | INTERACTS WITH Aflatoxin B2 alpha; benzo[e]pyrene; methapyrilene | 3 | 185162901 | 185191955 | Human | 3 | symbol , COSMIC , name , Human Proteome Map | gene, ncrna, VALIDATED [RefSeq] |
| 12693639 | LOC101954746 | peroxisomal bifunctional enzyme | ENCODES a protein that exhibits (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity (ortholog); intramolecular oxidoreductase activity, transposing C=C bonds (ortholog); INVOLVED IN fatty acid beta-oxidation (ortholog); PARTICIPATES IN valproic acid pharmacokinetics pathway; ASSOCIATED WITH cholest asis (ortholog); chronic kidney disease (ortholog); Fanconi renotubular syndrome 1 (ortholog); FOUND IN cytosol (ortholog) | NW_004936578 | 4554164 | 4666427 | Squirrel | 52 | old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |