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Identification
HMDB Protein ID HMDBP00380
Secondary Accession Numbers
  • 5617
  • HMDBP04498
Name Peroxisomal bifunctional enzyme
Synonyms
  1. 3-hydroxyacyl-CoA dehydrogenase
  2. Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase
  3. PBE
  4. PBFE
Gene Name EHHADH
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Not Available
Pathways
  • beta-Alanine metabolism
  • Butanoate metabolism
  • fatty acid beta-oxidation
  • fatty acid metabolism
  • Lysine degradation
  • Peroxisome
  • PPAR signaling pathway
  • Propanoate metabolism
  • Tryptophan metabolism
  • Valine, leucine and isoleucine degradation
  • Valproic Acid Metabolism Pathway
Reactions
(3S)-3-hydroxyacyl-CoA → trans-2(or 3)-enoyl-CoA + Water details
(3Z)-dodec-3-enoyl-CoA → (2E)-Dodecenoyl-CoA details
(S)-3-hydroxyacyl-CoA + NAD → 3-oxoacyl-CoA + NADH details
3-Hydroxybutyryl-CoA + NAD → Acetoacetyl-CoA + NADH + Hydrogen Ion details
3-Hydroxybutyryl-CoA → (E)-but-2-enoyl-CoA + Water details
3-Hydroxypropionyl-CoA → Acrylyl-CoA + Water details
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Water details
(S)-3-Hydroxydodecanoyl-CoA → (2E)-Dodecenoyl-CoA + Water details
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Ion details
2-Methyl-3-hydroxybutyryl-CoA → Tiglyl-CoA + Water details
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoA details
(S)-3-Hydroxyhexadecanoyl-CoA + NAD → 3-Oxohexadecanoyl-CoA + NADH + Hydrogen Ion details
(S)-3-Hydroxyhexadecanoyl-CoA → (2E)-Hexadecenoyl-CoA + Water details
(S)-3-Hydroxytetradecanoyl-CoA + NAD → 3-Oxotetradecanoyl-CoA + NADH details
(S)-3-Hydroxytetradecanoyl-CoA → (2E)-Tetradecenoyl-CoA + Water details
(S)-3-Hydroxydodecanoyl-CoA + NAD → 3-Oxododecanoyl-CoA + NADH + Hydrogen Ion details
(S)-Hydroxydecanoyl-CoA + NAD → 3-Oxodecanoyl-CoA + NADH + Hydrogen Ion details
(S)-Hydroxydecanoyl-CoA → (2E)-Decenoyl-CoA + Water details
(S)-Hydroxyoctanoyl-CoA + NAD → 3-Oxooctanoyl-CoA + NADH + Hydrogen Ion details
(S)-Hydroxyoctanoyl-CoA → (2E)-Octenoyl-CoA + Water details
(S)-Hydroxyhexanoyl-CoA + NAD → 3-Oxohexanoyl-CoA + NADH + Hydrogen Ion details
(S)-Hydroxyhexanoyl-CoA → trans-2-Hexenoyl-CoA + Water details
cis,cis-3,6-Dodecadienoyl-CoA → trans,cis-Lauro-2,6-dienoyl-CoA details
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Ion details
(E)-but-2-enoyl-CoA + Water → 3-Hydroxybutyryl-CoA details
(3S)-3-Hydroxyadipyl-CoA + NAD → 3-Oxoadipyl-CoA + NADH + Hydrogen Ion details
5-Carboxy-2-pentenoyl-CoA + Water → (3S)-3-Hydroxyadipyl-CoA details
GO Classification
Biological Process
fatty acid beta-oxidation
internal protein amino acid acetylation
Cellular Component
mitochondrion
nucleus
peroxisome
Function
binding
catalytic activity
coenzyme binding
cofactor binding
oxidoreductase activity
Molecular Function
3-hydroxyacyl-CoA dehydrogenase activity
enoyl-CoA hydratase activity
nucleotide binding
coenzyme binding
dodecenoyl-CoA delta-isomerase activity
Process
metabolic process
cellular metabolic process
organic acid metabolic process
oxoacid metabolic process
carboxylic acid metabolic process
monocarboxylic acid metabolic process
fatty acid metabolic process
Cellular Location
  1. Peroxisome
Gene Properties
Chromosome Location 3
Locus 3q26.3-q28
SNPs EHHADH
Gene Sequence
>2172 bp
ATGGCCGAGTATACGCGGCTGCACAACGCCTTGGCGCTAATCCGCCTCCGAAACCCGCCG
GTCAACGCGATCAGTACGACTTTACTCCGTGATATAAAAGAAGGACTACAGAAAGCTGGA
AGAGACCATACAATAAAAGCCATTGTGATTTGTGGAGCAGAGGGCAAATTTTCTGCAGGT
GCTGATATTCGTGGCTTCAGTGCTCCTAGGACATTTGGCCTTATACTGGGACATGTAGTA
GATGAAATACAGAGAAATGAGAAGCCCGTGGTGGCAGCAATCCAAGGCATGGCTTTCGGA
GGGGGACTAGAGCTGGCCCTGGGCTGTCACTATAGGATTGCCCACGCAGACGCTCAAGTT
GGCTTACCAGAAGTTACACTTGGACTTCTCCCTGGTGCAAGAGGAACCCAGCTTCTCCCC
AGACTCACTGGAGTTCCTGCTGCACTTGACTTAATTACCTCAGGAAGACGTATTTTAGCA
GATGAAGCACTCAAGCTGGGCATTCTAGATAAAGTTGTAAACTCAGACCCGGTTGAAGAA
GCAATCAGATTTGCTCAGAGAGTTTCAGATCAACCTCTAGAATCCCGTAGACTCTGCAAC
AAGCCAATTCAGAGCTTGCCCAACATGGACAGCATTTTTAGTGAGGCCCTCTTGAAGATG
CGGAGGCAGCACCCTGGGTGTCTTGCACAGGAGGCTTGTGTCCGTGCAGTCCAGGCTGCT
GTGCAGTATCCCTATGAAGTGGGCATCAAGAAGGAGGAGGAGCTGTTTCTATATCTTTTG
CAATCAGGGCAGGCTAGAGCCCTGCAATATGCTTTCTTCGCTGAAAGGAAAGCAAATAAG
TGGTCAACTCCCTCCGGAGCATCGTGGAAAACAGCATCAGCGCGGCCTGTCTCCTCAGTT
GGTGTTGTTGGCTTGGGAACAATGGGCCGAGGCATTGTCATTTCTTTTGCAAGGGCCAGG
ATTCCTGTGATTGGTGTAGACTCGGACAAAAACCAGCTAGCAACTGCAAACAAGATGATA
ACCTCTGTCTTGGAAAAAGAAGCCTCCAAAATGCAACAGAGCGGCCACCCTTGGTCAGGA
CCAAAACCCAGGTTAACTTCATCTGTGAAGGAGCTTGGTGGTGTAGATTTAGTCATTGAA
GCAGTATTTGAGGAAATGAGCCTGAAGAAGCAGGTCTTTGCTGAACTCTCAGCTGTGTGC
AAACCAGAAGCATTTTTGTGCACTAATACTTCAGCCCTGGATGTTGATGAGATTGCTTCT
TCCACTGATCGTCCTCACTTGGTCATTGGCACCCACTTCTTTTCGCCAGCTCATGTCATG
AAGTTGTTAGAGGTTATTCCCAGCCAATACTCTTCCCCCACTACCATTGCCACTGTTATG
AACTTATCAAAAAAGATTAAAAAGATTGGAGTCGTTGTAGGCAACTGTTTTGGATTTGTG
GGGAATCGAATGTTGAATCCTTACTACAATCAGGCATATTTCTTGTTAGAAGAAGGCAGC
AAACCAGAGGAGGTAGATCAGGTGCTGGAAGAGTTTGGTTTTAAAATGGGACCTTTTAGA
GTGTCTGATCTTGCTGGGTTGGATGTGGGCTGGAAATCTAGAAAGGGGCAAGGTCTTACT
GGACCTACATTGCTTCCAGGAACTCCTGCCCGAAAAAGGGGTAATAGGAGGTACTGCCCA
ATTCCTGATGTGCTCTGTGAATTAGGACGATTTGGCCAGAAGACAGGTAAGGGTTGGTAT
CAATATGACAAGCCATTGGGTAGGATTCACAAACCTGATCCCTGGCTTTCCACATTCCTA
TCACGGTATAGAAAACCCCATCACATTGAACCACGTACCATTAGCCAGGATGAGATCCTT
GAACGCTGCTTATATTCACTTATCAATGAAGCATTCCGTATCTTGGGAGAAGGGATAGCT
GCTAGCCCAGAGCACATTGATGTTGTCTATTTACATGGATATGGATGCGCAAGGCACAAG
GGCGGGCCCATGTTCTATGCTTCCACAGTTGGGTTGCCCACAGTTCTAGAGAAATTGCAG
AAATATTACAGGCAGAACCCTGATATTCCCCAACTGGAGCCAAGTGACTATCTAAAAAAA
CTGGCTTCTCAGGGAAACCCTCCCCTGAAAGAATGGCAAAGCTTGGCAGGCTCCCCTAGC
AGTAAATTGTGA
Protein Properties
Number of Residues 723
Molecular Weight 69153.26
Theoretical pI 9.057
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Peroxisomal bifunctional enzyme
MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAG
ADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQV
GLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEE
AIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAA
VQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSV
GVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSG
PKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIAS
STDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFV
GNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLT
GPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFL
SRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHK
GGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPS
SKL
GenBank ID Protein 452045
UniProtKB/Swiss-Prot ID Q08426
UniProtKB/Swiss-Prot Entry Name ECHP_HUMAN
PDB IDs Not Available
GenBank Gene ID L07077
GeneCard ID EHHADH
GenAtlas ID EHHADH
HGNC ID HGNC:3247
References
General References
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  3. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
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