LMPD Database

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LMP002229

UniProt Annotations

Entry Information
Gene Nameenoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase
Protein EntryECHP_HUMAN
UniProt IDQ08426
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q08426-1; Sequence=Displayed; Name=2; IsoId=Q08426-2; Sequence=VSP_042811;
Catalytic Activity(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl- CoA + H(2)O.
Catalytic Activity(3Z)-dodec-3-enoyl-CoA = (2E)-dodec-2-enoyl- CoA.
Catalytic Activity(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.
DiseaseFanconi renotubular syndrome 3 (FRTS3) [MIM
Enzyme RegulationEnzyme activity enhanced by acetylation.
MiscellaneousAbsent in patients suffering with peroxisomal disorders such as Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease.
PathwayLipid metabolism; fatty acid beta-oxidation.
PtmAcetylated, leading to enhanced enzyme activity. Acetylation is enhanced by up to 80% after treatment either with trichostin A (TSA) or with nicotinamide (NAM) with highest increase on Lys-346. Acetylation and enzyme activity increased by about 1.5% on addition of fatty acids. {ECO
SimilarityIn the C-terminal section; belongs to the 3- hydroxyacyl-CoA dehydrogenase family.
SimilarityIn the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.
Subcellular LocationPeroxisome.
SubunitMonomer.
Tissue SpecificityLiver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain. {ECO