ADHFE1 - Hydroxyacid-oxoacid transhydrogenase, mitochondrial - human protein (Function)
 
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Protein
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References

 
ADHFE1 »  Hydroxyacid-oxoacid transhydrogenase, mitochondrial   [ EC 1.1.99.24 ]  (HOT)
 
Protein also known as:  Alcohol dehydrogenase iron-containing protein 1 (ADHFe1).
Gene name:  ADHFE1
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 21 4
GENE REF ISO

Function

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Overview 
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.  
1
  • CuratedUniProtKB
GO molecular function 
Hydroxyacid-oxoacid transhydrogenase activitydefinition[GO:0047988]  
1
  • IDAUniProtKB
Metal ion bindingdefinition[GO:0046872] silver  
  • IEAInterPro 2 GO
GO biological process 
Molecular hydrogen transportdefinition[GO:0015993]  
1
  • IDAUniProtKB
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reactions
(S)-3-hydroxybutanoate + 2-oxoglutarate acetoacetate + (R)-2-hydroxyglutarate.  
1
  • CuratedUniProtKB
4-hydroxybutanoate + 2-oxoglutarate succinic semialdehyde + (R)-2-hydroxyglutarate.  
1
  • CuratedUniProtKB
Pathways 
According to Reactome, this protein belongs to the following pathway:
Metabolism  REACT_111217  
 

Biophysicochemical properties

Kinetic parameters
KM 0.04 mM for SSA
KM 0.8 mM for L-3-OHB
KM 1.2 mM for 2-KG
KM 0.17 mM for GHB
KM 0.12 mM for D-2-HG
Vmax 16 nmol/h/mg enzyme with GHB and 2-KG as substrates
Vmax 1.8 nmol/h/mg enzyme with L-3-OHB and 2-KG as substrates
Vmax 0.5 nmol/h/mg enzyme with SSA and D-2-HG as substrates
Dependence
pH Optimum pH is 7.5.

Keywords

Molecular function 
Oxidoreductase  definition   [KW-0560]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GenomeRNAi: 137872
PRO: PR:Q8IWW8