HuLPr24799

Uniprot ID

HOT_HUMAN

Uniprot Acc

Q8IWW8;B4DTJ8;Q49A19;Q68CI7;Q6P2B6;Q96MF9;

Protein name

Hydroxyacid-oxoacid transhydrogenase, mitochondrial

Comment

FUNCTION: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.||CATALYTIC ACTIVITY:(S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate.||CATALYTIC ACTIVITY:2-oxoglutaric acid + 4-hydroxybutanoic acid = (R)-2-hydroxyglutaric acid + succinic semialdehyde.||BIOPHYSICOCHEMICAL PROPERTIES:Kinetic parameters: KM=0.17 mM for GHB; KM=1.2 mM for 2-KG; KM=0.12 mM for D-2-HG; KM=0.04 mM for SSA; KM=0.8 mM for L-3-OHB; Vmax=16 nmol/h/mg enzyme with GHB and 2-KG as substrates; Vmax=0.5 nmol/h/mg enzyme with SSA and D-2-HG as substrates; Vmax=1.8 nmol/h/mg enzyme with L-3-OHB and 2-KG as substrates; pH dependence: Optimum pH is 7.5;||SUBCELLULAR LOCATION:Mitochondrion (By similarity).||ALTERNATIV

Subcellular localization

Mitochondrion(By similarity).

Gene name

alcohol dehydrogenase, iron containing, 1

Protein sequence

MAAAARARVAYLLRQLQRAACQCPTHSHTYSQAPGLSPSG KTTDYAFEMAVSNIRYGAAVTKEVGMDLKNMGAKNVCLM TDKNLSKLPPVQVAMDSLVKNGIPFTVYDNVRVEPTDSS FMEAIEFAQKGAFDAYVAVGGGSTMDTCKAANLYASSPH SDFLDYVSAPIGKGKPVSVPLKPLIAVPTTSGTGSETTG VAIFDYEHLKVKIGITSRAIKPTLGLIDPLHTLHMPARV VANSGFDVLCHALESYTTLPYHLRSPCPSNPITRPAYQG SNPISDIWAIHALRIVAKYLKRAVRNPDDLEARSHMHLA SAFAGIGFGNAGVHLCHGMSYPISGLVKMYKAKDYNVDH PLVPHGLSVVLTSPAVFTFTAQMFPERHLEMAEILGADT RTARIQDAGLVLADTLRKFLFDLDVDDGLAAVGYSKADI PALVKGTLPQERVTKLAPCPQSEEDLAALFEASMKLY

Database cross reference

RefSeq Protein accession:NP_653251
RefSeq Protein gi:133922590

HLPP validation

Yes/No

Yes

Project name

Chinese Liver;French Liver;Human Fetal Liver;Human Liver Organelles;

GO:0005759;C:mitochondrial matrix;TAS:Reactome.

GO-F

GO:0047988;F:hydroxyacid-oxoacid transhydrogenase activity;IDA:UniProtKB. GO:0046872;F:metal ion binding;IEA:InterPro.

GO-P

GO:0006103;P:2-oxoglutarate metabolic process;TAS:Reactome. GO:0015993;P:molecular hydrogen transport;IDA:UniProtKB. GO:0055114;P:oxidation-reduction process;IEA:UniProtKB-KW.