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3-hydroxybutyrate dehydrogenase, type 1
Symbols BDH1 ; BDH; SDR9C1
External IDs OMIM603063 MGI1919161 HomoloGene20860 GeneCards: BDH1 Gene
EC number
RNA expression pattern
PBB GE BDH1 211715 s at tn.png
More reference expression data
Species Human Mouse
Entrez 622 71911
Ensembl ENSG00000161267 ENSMUSG00000046598
UniProt Q02338 Q80XN0
RefSeq (mRNA) NM_004051 NM_001122683
RefSeq (protein) NP_004042 NP_001116155
Location (UCSC) Chr 3:
197.51 – 197.57 Mb
Chr 16:
31.42 – 31.46 Mb
PubMed search [1] [2]

D-beta-hydroxybutyrate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the BDH1 gene.[1][2][3]

This gene encodes a member of the short-chain dehydrogenase/reductase gene family. The encoded protein forms a homotetrameric lipid-requiring enzyme of the mitochondrial membrane and has a specific requirement for phosphatidylcholine for optimal enzymatic activity. The encoded protein catalyzes the interconversion of acetoacetate and (R)-3-hydroxybutyrate, the two major ketone bodies produced during fatty acid catabolism. Alternatively spliced transcript variants encoding the same protein have been described.[3]

See also[edit]


  1. ^ Marks AR, McIntyre JO, Duncan TM, Erdjument-Bromage H, Tempst P, Fleischer S (Aug 1992). "Molecular cloning and characterization of (R)-3-hydroxybutyrate dehydrogenase from human heart". J Biol Chem 267 (22): 15459–63. PMID 1639787. 
  2. ^ Persson B, Kallberg Y, Bray JE, Bruford E, Dellaporta SL, Favia AD, Duarte RG, Jornvall H, Kavanagh KL, Kedishvili N, Kisiela M, Maser E, Mindnich R, Orchard S, Penning TM, Thornton JM, Adamski J, Oppermann U (Feb 2009). "The SDR (short-chain dehydrogenase/reductase and related enzymes) nomenclature initiative". Chem Biol Interact 178 (1-3): 94–8. doi:10.1016/j.cbi.2008.10.040. PMC 2896744. PMID 19027726. 
  3. ^ a b "Entrez Gene: BDH1 3-hydroxybutyrate dehydrogenase, type 1". 

Further reading[edit]