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

Three steps

• CHEBI:28701Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 3 morecytosol, peroxisomeOne of 7 routes

• 17alpha-hydroxypregnenoloneReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxypregnenolone)

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

• chlorine(.)Reach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and formate (cytosol to extracellular)
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 1 moreextracellular space, cytosolOne of 2 routes

• glycocholateReach 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. glycocholate(out) + glutarate(in) = glycocholate(in) + glutarate(out)

  Catalysed by SUGCT, SLC22A24inner side of the membrane, outer side of the membraneOne of 2 routes

• adipic acidReach 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 4 routes

• 3-hydroxypyruvic acidReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. L-Serine:pyruvate aminotransferase

  Catalysed by HMGCL, HMGCLL1, AGXTmitochondrial matrix, cytosolOne of 2 routes

• 3-oxopropanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosol

• D-serineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. D-serine = pyruvate + NH4(+)

  Catalysed by HMGCL, HMGCLL1, SRRmitochondrial matrix, cytosol

• taurocholateReach 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. taurocholate(out) + glutarate(in) = taurocholate(in) + glutarate(out)

  Catalysed by SUGCT, SLC22A24, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:36882Reach 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 hydro-lyase (itaconyl-CoA-forming)

  Catalysed by SUGCT, AUHmitochondrial matrix

• (R)-lactic acidReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by HMGCL, HMGCLL1, LDHDmitochondrial matrix, cytosol

• 2-iminopropanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by HMGCL, HMGCLL1, RIDAmitochondrial matrix, cytosol

• phosphoenolpyruvic acidReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. pyruvate kinase (dTDP)

  Catalysed by HMGCL, HMGCLL1, PKM, PKLRmitochondrial matrix, cytosol

• 13-cis-retinalReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 2 moreextracellular space, cytosol, endoplasmic reticulum

• N-acetyl-beta-neuraminic acidReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by HMGCL, HMGCLL1, NPLmitochondrial matrix, cytosol

• N-(acetyl)-sphing-4-enineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

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

• taurineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

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

• a 3-acylpyruvateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by HMGCL, HMGCLL1, FAHD1mitochondrial matrix, cytosol

• glycineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 3 morecytosol, mitochondrial matrixOne of 10 routes

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

  Catalysed by SUGCT, OXCT1, HADH, HSD17B10One of 3 routes

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

  Catalysed by SUGCT, OXCT1, OXCT2, EHHADH and 2 more

• (R)-methylmalonyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 1 morecytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. acid phosphatase 2, lysosomal (CoA)

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

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

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

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

• (6Z,9Z,12Z)-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 (gamma-linolenate)

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

• butanoyl-CoAReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and butyrate (cytosol to extracellular)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 9 moreextracellular space, cytosolOne of 11 routes

• dodecanoyl-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 (lauric acid)

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

• eicosanoyl-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 (eicosanoate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 12 routes

• (9Z,12Z)-octadecadienoyl-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)-octadecadienoic acid)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 8 morecytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

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

• octanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 1 morecytosol, mitochondrial matrixOne of 20 routes

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

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 5 routes

• octadecanoyl-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 (stearate)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 7 morecytosolOne of 15 routes

• prostaglandin F2alphaReach 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 F2alpha(out) + glutarate(in) = prostaglandin F2alpha(in) + glutarate(out)

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

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate

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

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by HMGCL, HMGCLL1, MPSTmitochondrial matrix, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosol

• sphing-4-enineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 4 morecytosol, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. diamine N-acetyltransferase (N1-acetylspermidine)

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

• L-methionineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by HMGCL, HMGCLL1, TYW1B, TYW1mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by SUGCT, SLC22A24, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• L-alanineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosolOne of 19 routes

• L-asparagineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. N-acetylglutamate synthase (N-acetyl-L-asparagine)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 1 morecytosol, mitochondrial matrix

• porphobilinogenReach from here →
  1. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  2. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA
  3. 2 5-aminolevulinate = porphobilinogen + 2 H2O + H(+)

  Catalysed by SUGCT, ALAS1, ALAS2, ALAD

• L,L-cystathionineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 1 morecytosol, mitochondrial matrix

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

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

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

  Catalysed by SUGCT, OXCT1, OXCT2, ACAA1 and 3 moreOne of 3 routes

• kynurenateReach 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. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)

  Catalysed by SUGCT, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes