(R)-2-hydroxyglutarate

Three steps

• sn-glycerol 3-phosphocholineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + AH2 + O2 = 2-[(11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + A + H2O
  3. 2-[(11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + H2O = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + sn-glycerol 3-phosphocholine + H(+)

  Catalysed by D2HGDH, PTGS2, PLA2G4AOne of 3 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. (R)-3-Hydroxybutanoate:NAD+ oxidoreductase

  Catalysed by ADHFE1, BDH1, BDH2mitochondrial matrixOne of 4 routes

• L-isoleucineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and isoleucine (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC7A5extracellular space, cytosolOne of 6 routes

• CHEBI:17203Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and proline (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC7A5extracellular space, cytosolOne of 6 routes

• all-trans-retinolReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by D2HGDH, AIFM1, PYROXD1, RETSATOne of 2 routes

• L-cysteineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by D2HGDH, AIFM1, PYROXD1, MOCOSOne of 19 routes

• diiodineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by D2HGDH, DIO1, DIO3, TPOcytosolOne of 2 routes

• nitrateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. 2 nitrite + AH2 + 2 H(+) = 2 nitric oxide + A + 2 H2O
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

  Catalysed by D2HGDH, SUOX, CYGB

• 3,3',5-triiodo-L-thyronine sulfateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. 3,3',5-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2
  3. aryl sulfotransferase (PAPS)

  Catalysed by D2HGDH, DIO1, DIO2, SULT1A3 and 3 morecytosolOne of 2 routes

• Ala-tRNA(Ala)Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by D2HGDH, SCLY, AARS1cytosol

• CHEBI:17895Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and tyrosine (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC7A5extracellular space, cytosolOne of 8 routes

• 7alpha-hydroxycholest-4-en-3-oneReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. Cholest-5-Ene-3Beta, 7Alpha-Diol:NAD+ 3-Oxidoreductase Bile Acid Biosynthesis

  Catalysed by D2HGDH, CH25H, HSD3B7endoplasmic reticulum

• CHEBI:18019Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and lysine (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC7A6extracellular space, cytosolOne of 4 routes

• 4-fumarylacetoacetateReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by ADHFE1, FAHmitochondrial matrix

• L-glutamineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and glutamine (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC3A2, SLC7A5 and 1 moreextracellular space, cytosolOne of 12 routes

• CHEBI:18095Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. Transport of Hydroxy-Proline

  Catalysed by D2HGDH, SCLY, SLC1A4extracellular space, cytosol

• S-methylmercury-L-cysteineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by D2HGDH, CDK5RAP1, SLC7A8inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-aminoisobutanoateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by D2HGDH, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by D2HGDH, SCLY, AGXT2

• pyridoxine 5'-phosphateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by D2HGDH, SCLYcytosol

• hypoioditeReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by D2HGDH, DIO1, DIO3, MPO and 1 more

• chlorine(.)Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and chloride (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC6A14extracellular space, cytosolOne of 5 routes

• L-aspartateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by D2HGDH, SCLY, AGXT2One of 3 routes

• acetateReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. transport of acetate and acetoacetate (cytosol to extracellular)

  Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A6 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• formic acidReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. transport of acetoacetate and formate (cytosol to extracellular)

  Catalysed by ADHFE1, SLC16A4, SLC16A5mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• propionic acidReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. transport of acetoacetate and propanoate (cytosol to extracellular)

  Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A7 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• butyric acidReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. transport of acetoacetate and butyrate (cytosol to extracellular)

  Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A7 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by D2HGDH, CDK5RAP1, IL4I1cytosol

• D-cysteineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by D2HGDH, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by D2HGDH, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 6 routes

• 7alpha,25-dihydroxycholesterolReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. cytochrome P450 (25-hydroxycholesterol)

  Catalysed by D2HGDH, CH25H, CYP7B1endoplasmic reticulumOne of 2 routes

• (S)-lactic acidReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. transport of acetoacetate and L-lactate (cytosol to extracellular)

  Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A7 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by ADHFE1, HMGCL, HMGCLL1mitochondrial matrix

• L-ornithineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and ornithine (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC7A6extracellular space, cytosolOne of 5 routes

• gamma-Glu-AlaReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by D2HGDH, SCLY, GGT1, GGT5 and 4 moreextracellular space, cytosol

• malonaldehydeReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. thromboxane A synthase (12(S)-HHT)

  Catalysed by D2HGDH, PTGS1, PTGS2, TBXAS1cytosolOne of 2 routes

• acetoacetyl-CoAReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by ADHFE1, AACSmitochondrial matrixOne of 2 routes

• glycineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-Alanine:glyoxylate aminotransferase

  Catalysed by D2HGDH, SCLY, AGXT, AGXT2mitochondrial matrixOne of 14 routes

• prostaglandin E1Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. prostaglandin G1 + AH2 = prostaglandin H1 + A + H2O
  3. prostaglandin-E synthase (prostaglandin E1)

  Catalysed by D2HGDH, PTGS2, PTGES, PTGES3 and 1 morecytosolOne of 2 routes

• prostaglandin I2Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin I2 synthase (prostaglandin H2)

  Catalysed by D2HGDH, PTGS1, PTGS2, PTGISendoplasmic reticulumOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin H2 + [thioredoxin]-dithiol = prostaglandin F2alpha + [thioredoxin]-disulfide

  Catalysed by D2HGDH, PTGS1, PTGS2, PRXL2BOne of 2 routes

• prostaglandin D2Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin-D synthase (prostaglandin H2)

  Catalysed by D2HGDH, PTGS1, PTGS2, HPGDS and 1 morecytosolOne of 3 routes

• 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O
  3. (15S)-15-Hydroxy-5,8,11-cis-13-trans-icosatetraenoate:NADP+ 15-oxidoreductase

  Catalysed by D2HGDH, TBXAS1, PTGS2, PTGIScytosolOne of 3 routes

• D-alanineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by D2HGDH, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• thromboxane A2Reach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. thromboxane A synthase (prostaglandin H2)

  Catalysed by D2HGDH, PTGS1, PTGS2, TBXAS1endoplasmic reticulumOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by D2HGDH, AIFM1, PYROXD1, MTO1

• D-threonineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by D2HGDH, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• L-tryptophanReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. transport of alanine and tryptophan (cytosol to extracellular)

  Catalysed by D2HGDH, SCLY, SLC7A5extracellular space, cytosolOne of 8 routes

• L-threonineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by D2HGDH, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 4 routes

• L-kynurenineReach from here →
  1. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by D2HGDH, SCLY, KYNU