3-hydroxy-3-methylglutarate

Three steps

• acetateReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. transport of acetate and acetoacetate (cytosol to extracellular)

  Catalysed by SUGCT, HMGCL, HMGCLL1, SLC16A4 and 4 moreextracellular space, cytosolOne of 2 routes

• formic acidReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. transport of acetoacetate and formate (cytosol to extracellular)

  Catalysed by SUGCT, HMGCL, HMGCLL1, SLC16A4 and 1 moreextracellular space, cytosolOne of 2 routes

• propionic acidReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. transport of acetoacetate and propanoate (cytosol to extracellular)

  Catalysed by SUGCT, HMGCL, HMGCLL1, SLC16A4 and 4 moreextracellular space, cytosolOne of 2 routes

• butyric acidReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. transport of acetoacetate and butyrate (cytosol to extracellular)

  Catalysed by SUGCT, HMGCL, HMGCLL1, SLC16A4 and 4 moreextracellular space, cytosolOne of 2 routes

• pentanoateReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. estrone 3-sulfate(out) + glutarate(in) = estrone 3-sulfate(in) + glutarate(out)
  3. pentanoate(in) + estrone 3-sulfate(out) = pentanoate(out) + estrone 3-sulfate(in)

  Catalysed by SUGCT, SLC22A24, SLC22A8, SLC22A11 and 1 moreinner side of the membrane, outer side of the membraneOne of 4 routes

• nicotinateReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. nicotinate(in) + urate(out) = nicotinate(out) + urate(in)

  Catalysed by SUGCT, SLC22A8, SLC22A12, SLC22A13inner side of the membrane, outer side of the membrane

• 5-aminolevulinateReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by SUGCT, ALAS1, ALAS2

• (S)-lactic acidReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. transport of acetoacetate and L-lactate (cytosol to extracellular)

  Catalysed by SUGCT, HMGCL, HMGCLL1, SLC16A4 and 4 moreextracellular space, cytosolOne of 3 routes

• O-propanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. propanoyl-CoA + (R)-carnitine = O-propanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CRATinner side of the membrane, outer side of the membraneOne of 3 routes

• acetoacetyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. acetoacetate + succinyl-CoA = acetoacetyl-CoA + succinate

  Catalysed by SUGCT, OXCT1One of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  3. glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)

  Catalysed by SUGCT, GCDHmitochondrial matrixOne of 2 routes

• (2E)-glutaconyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by SUGCT, ACOX1

• itaconyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. itaconate + succinyl-CoA = itaconyl-CoA + succinate

  Catalysed by SUGCTOne of 6 routes

• 15-oxoprostaglandin E2Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. prostaglandin E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)
  3. prostaglandin E2 + NAD(+) = 15-oxoprostaglandin E2 + NADH + H(+)

  Catalysed by SUGCT, SLC22A8, HPGDinner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin H2Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. prostaglandin E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)
  3. prostaglandin E synthase (prostaglandin E2)

  Catalysed by SUGCT, SLC22A8, PTGES, PTGES3 and 1 moreinner side of the membrane, outer side of the membrane, endoplasmic reticulumOne of 4 routes

• prostaglandin D2Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. prostaglandin F2alpha(out) + glutarate(in) = prostaglandin F2alpha(in) + glutarate(out)
  3. aldo-keto reductase (prostaglandin D2)

  Catalysed by SUGCT, SLC22A8, AKR1C3inner side of the membrane, outer side of the membrane, cytosol

• O-acetyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. (R)-carnitine + acetyl-CoA = O-acetyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CRATinner side of the membrane, outer side of the membraneOne of 3 routes

• glutathioneReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. taurocholate(out) + glutarate(in) = taurocholate(in) + glutarate(out)
  3. taurocholate(in) + glutathione(in) + ATP + H2O = taurocholate(out) + glutathione(out) + ADP + phosphate + H(+)

  Catalysed by SUGCT, SLC22A24, SLC22A8, ABCC4inner side of the membrane, outer side of the membraneOne of 3 routes

• CHEBI:730073Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. Peroxidase

  Catalysed by SUGCT, SLC22A8, MPOinner side of the membrane, outer side of the membrane, nucleusOne of 3 routes

• hexanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  3. hexanedioate + glutaryl-CoA = hexanedioyl-CoA + glutarate

  Catalysed by SUGCTOne of 3 routes

• O-dodecanoyl-R-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. dodecanoyl-CoA + (R)-carnitine = O-dodecanoyl-R-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-tetradecanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-octadecanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. (R)-carnitine + octadecanoyl-CoA = O-octadecanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-eicosanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. eicosanoyl-CoA + (R)-carnitine = O-eicosanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-(9Z)-octadecenoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-4,8-dimethylnonanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. 4,8-dimethylnonanoyl-CoA + (R)-carnitine = O-4,8-dimethylnonanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2, CRAT and 1 moreinner side of the membrane, outer side of the membrane

• O-hexanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. hexanoyl-CoA + (R)-carnitine = O-hexanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CRATinner side of the membrane, outer side of the membrane

• O-3-oxobutanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. acetoacetyl-CoA + (R)-carnitine = O-3-oxobutanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CRATinner side of the membrane, outer side of the membrane

• 16alpha-hydroxydehydroepiandrosterone 3-sulfateReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. dehydroepiandrosterone 3-sulfate(out) + glutarate(in) = dehydroepiandrosterone 3-sulfate(in) + glutarate(out)
  3. dehydroepiandrosterone 3-sulfate + reduced [NADPH--hemoprotein reductase] + O2 = 16alpha-hydroxydehydroepiandrosterone 3-sulfate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by SUGCT, SLC22A24, SLC22A8, CYP3A7inner side of the membrane, outer side of the membrane

• a 3-oxoacyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. a 3-oxo acid + succinyl-CoA = a 3-oxoacyl-CoA + succinate

  Catalysed by SUGCT, OXCT1, OXCT2