EHHADH - Peroxisomal bifunctional enzyme - human protein (Function)
 
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References

 
EHHADH »  Peroxisomal bifunctional enzyme  (PBE)
 
Gene name:  EHHADH
Family names: In the N-terminal section Enoyl-CoA hydratase/isomerase
In the C-terminal section 3-hydroxyacyl-CoA dehydrogenase
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 31 2
GENE REF ISO

Function

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GO molecular function 
3-hydroxyacyl-CoA dehydrogenase activitydefinition[GO:0003857]  
1
  • NASUniProtKB
Coenzyme bindingdefinition[GO:0050662] silver  
  • IEAInterPro 2 GO
Dodecenoyl-CoA delta-isomerase activitydefinition[GO:0004165]  
  • IEAEC 2 GO
Enoyl-CoA hydratase activitydefinition[GO:0004300]  
1
  • NASUniProtKB
Enzyme bindingdefinition[GO:0019899]  
1
  • IPIUniProtKB
Receptor bindingdefinition[GO:0005102]  
1
  • IPIUniProtKB
GO biological process 
Fatty acid beta-oxidationdefinition[GO:0006635]  
1
  • NASUniProtKB
  • IEAUniPathway
Internal protein amino acid acetylationdefinition[GO:0006475]  
1
  • IDAUniProtKB
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reactions
EC 4.2.1.17: (3S)-3-hydroxyacyl-CoA trans-2(or 3)-enoyl-CoA + H(2)O.  
  • CuratedUniProtKB
EC 5.3.3.8: (3Z)-dodec-3-enoyl-CoA (2E)-dodec-2-enoyl-CoA.  
  • CuratedUniProtKB
EC 1.1.1.35: (S)-3-hydroxyacyl-CoA + NAD(+) 3-oxoacyl-CoA + NADH.  
  • CuratedUniProtKB
It is regulated in the following manner
Enzyme activity enhanced by acetylation.  
1
  • CuratedUniProtKB
Pathways 
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
beta-Alanine metabolism  hsa00410+1962  
Butanoate metabolism  hsa00650+1962  
Fatty acid metabolism  hsa00071+1962  
Lysine degradation  hsa00310+1962  
Metabolic pathways  hsa01100+1962  
Peroxisome  hsa04146+1962  
PPAR signaling pathway  hsa03320+1962  
Propanoate metabolism  hsa00640+1962  
Tryptophan metabolism  hsa00380+1962  
Valine, leucine and isoleucine degradation  hsa00280+1962  
 
More information is available from:
Note 
Absent in patients suffering with peroxisomal disorders such as Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease.  
  • CuratedUniProtKB
 

Keywords

Biological process 
Fatty acid metabolism  definition   [KW-0276]
Lipid metabolism  definition   [KW-0443]
Molecular function 
Isomerase  definition   [KW-0413]
Lyase  definition   [KW-0456]
Oxidoreductase  definition   [KW-0560]
Technical term 
Multifunctional enzyme  definition   [KW-0511]
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: Q08426
Other
GenomeRNAi: 1962
PRO: PR:Q08426