(3S)-3-hydroxy-3-methylglutaryl-CoA

Three steps

• N(8)-acetylspermidineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. N(8)-acetylspermidine + H2O = spermidine + acetate

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosol

• (3Z)-dodecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 3 morecytosol, mitochondrial matrixOne of 3 routes

• (3S)-citramalyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. (3S)-citramalyl-CoA = itaconyl-CoA + H2O

  Catalysed by SUGCT, AUHOne of 2 routes

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 2 morecytosol, endoplasmic reticulumOne of 3 routes

• an N-acetyl-L-cysteine-S-conjugateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. an N-acetyl-L-cysteine-S-conjugate + H2O = an S-substituted L-cysteine + acetate

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosol

• deoxycholoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 2 morecytosolOne of 4 routes

• estrone 3-sulfateReach 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. estrone 3-sulfate(out) + glutarate(in) = estrone 3-sulfate(in) + glutarate(out)

  Catalysed by SUGCT, SLC22A24, SLC22A8, SLC22A11inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach 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. prostaglandin E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)

  Catalysed by SUGCT, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• decanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 6 morecytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Carnitine O-Palmitoyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 11 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by HMGCL, HMGCLL1, HOGA1mitochondrial matrix, cytosol

• 3-hydroxyisovaleryl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1Bcytosol

• CHEBI:63544Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 9 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Carnitine O-Palmitoyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 8 routes

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by HMGCL, HMGCLL1, HOGA1mitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Activation of dodecanedioic acid

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Production of Sebacoylcarnitine

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Production of Suberylcarnitine

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1Bcytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 5 routes

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 2 morecytosol, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 4 morecytosol, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Production of Hexadecanedioylcarnitine

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. fatty-acid-CoA ligase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 1 morecytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 6 morecytosolOne of 3 routes