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

Three steps

• palmitoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Palmitate:CoA ligase (AMP-forming)

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

• oleoyl-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 octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1BcytosolOne of 11 routes

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

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

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

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 6 moreextracellular space, cytosolOne of 46 routes

• (S)-malateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (S)-malate + NAD(+) = pyruvate + CO2 + NADH

  Catalysed by HMGCL, HMGCLL1, ME2mitochondrial matrix, cytosolOne of 2 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and formate (cytosol to extracellular)
  3. Formate:tetrahydrofolate ligase (ADP-forming)

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

• CHEBI:15650Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 32 morecytosolOne of 2 routes

• CHEBI:15727Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

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

• CHEBI:15746Reach 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-acetylspermine)

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

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

  Catalysed by HMGCL, HMGCLL1, ADHFE1mitochondrial matrixOne of 2 routes

• 2-methyl-3-oxopropanoic acidReach 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, cytosol

• 11-cis-retinolReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 5 morecytosol

• (R)-carnitineReach 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. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)

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

• oxaloacetateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. Pyruvate:carbon-dioxide ligase (ADP-forming)

  Catalysed by HMGCL, HMGCLL1, PCmitochondrial matrix, cytosolOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

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

• (S)-lactateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by HMGCL, HMGCLL1, LDHA, LDHB and 3 moremitochondrial matrix, cytosol

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

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by HMGCL, HMGCLL1, KYAT1mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by HMGCL, HMGCLL1, FAHD1mitochondrial matrix, cytosol

• ferrocytochrome cReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and L-lactate (cytosol to extracellular)
  3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase

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

• 4-maleylacetoacetateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  3. 4-maleylacetoacetate = 4-fumarylacetoacetate

  Catalysed by HMGCL, HMGCLL1, FAH, GSTZ1

• 3-hydroxypyruvateReach 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 = 3-hydroxypyruvate + L-alanine

  Catalysed by HMGCL, HMGCLL1, AGXTmitochondrial matrix, cytosol

• itaconateReach 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. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate

  Catalysed by SUGCTOne of 5 routes

• 17alpha-hydroxyprogesteroneReach 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-hydroxyprogesterone)

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

• anilineReach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 2 morecytosol

• aceneuramateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by HMGCL, HMGCLL1, NPLmitochondrial matrix, cytosol

• CHEBI:17312Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

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

• 5'-deoxyadenosineReach 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, cytosol

• L-cysteineReach 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-cysteine)

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

• (7Z,10Z,13Z)-hexadecatrienoyl-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 (C18:3->C16:3), Mitochondrial

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 3 morecytosol, mitochondrial matrix

• 4-methylumbelliferyl acetateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5 and 6 moreextracellular space, cytosol

• urateReach 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. urate(in) + glutarate(out) = urate(out) + glutarate(in)

  Catalysed by SUGCT, SLC22A8inner side of the membrane, outer side of the membraneOne of 3 routes

• all-trans-retinalReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and retinoate (cytosol to extracellular)
  3. retinoate reduction

  Catalysed by HMGCL, HMGCLL1, SLC16A4, SLC16A5extracellular space, cytosol

• CHEBI:17927Reach from here →
  1. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
  2. Production of Glutaryl Carnitine
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1Bcytosol

• fumaric acidReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  3. 4-fumarylacetoacetate fumarylhydrolase

  Catalysed by HMGCL, HMGCLL1, FAHcytosolOne of 2 routes

• dTDPReach 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

• 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)Reach 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. 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(out) + glutarate(in) = 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(in) + glutarate(out)

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

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosol

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosol

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

  Catalysed by HMGCL, HMGCLL1, AGXT2mitochondrial matrix, cytosol

• (5Z)-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-7-enoyl-CoA)

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

• (11Z)-docosenoyl-CoA(4-)Reach 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-cetoleic acid)

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

• (7Z)-tetradecenoyl-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 ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-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 (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-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 Trans (C18:2->C16:2), Mitochondrial

  Catalysed by SUGCT, CPT1A, CPT1C, CPT1B and 3 morecytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-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 (C12:3->C10:2), Mitochondrial

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

• (5Z)-octenoyl-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 (C10:2->C8:1), Mitochondrial

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

• CHEBI:27540Reach 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 (hexanoic acid)

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

• hydroxyacetoneReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. Acetoacetate carboxy-lyase (acetone-forming)
  3. cytochrome P450 (acetone)

  Catalysed by HMGCL, HMGCLL1, CYP2E1mitochondrial matrix, cytosol

• elaidic acidReach 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 (elaidate)

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