itaconate

Three steps

• succinyl-4'-phosphopantetheineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. succinyl-CoA + H2O = succinyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCT and 3 moremitochondrial matrix

• estroneReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. estrone 3-sulfate(out) + glutarate(in) = estrone 3-sulfate(in) + glutarate(out)
  3. Estrone 3-sulfate sulfohydrolase

  Catalysed by SUGCT, SLC22A24, SLC22A8, SLC22A11 and 1 moreinner side of the membrane, outer side of the membrane, cytosolOne of 3 routes

• propanoateReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. estrone 3-sulfate(out) + glutarate(in) = estrone 3-sulfate(in) + glutarate(out)
  3. propanoate(in) + estrone 3-sulfate(out) = propanoate(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

• anilineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, NAT2 and 1 moremitochondrial matrix, cytosol

• CHEBI:17312Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrix, cytosolOne of 2 routes

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2, CPT1A and 1 moreinner side of the membrane, outer side of the membraneOne of 3 routes

• L-cysteineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, NAGSmitochondrial matrix

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 2 moremitochondrial matrix

• xanthineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. xanthine + NAD(+) + H2O = urate + NADH + H(+)

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

• glycine betaineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)

  Catalysed by SUGCT, SLC22A8, SLC22A5inner side of the membrane, outer side of the membraneOne of 2 routes

• 17beta-estradiol 17-O-(3-sulfo-beta-D-glucuronate)Reach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. 17beta-estradiol 17-O-(beta-D-glucuronate)(out) + glutarate(in) = 17beta-estradiol 17-O-(beta-D-glucuronate)(in) + glutarate(out)
  3. 17beta-estradiol 17-O-(beta-D-glucuronate) + 3'-phosphoadenylyl sulfate = 17beta-estradiol 17-O-(3-sulfo-beta-D-glucuronate) + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SUGCT, SLC22A24, CHST10inner side of the membrane, outer side of the membrane

• CHEBI:17927Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrix, cytosol

• butanoateReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. estrone 3-sulfate(out) + glutarate(in) = estrone 3-sulfate(in) + glutarate(out)
  3. butanoate(out) + estrone 3-sulfate(in) = butanoate(in) + estrone 3-sulfate(out)

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

• 5-hydroxyisourateReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. urate:oxygen oxidoreductase

  Catalysed by SUGCT, SLC22A8inner side of the membrane, outer side of the membrane, cytosol

• O-octanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. octanoyl-CoA + (R)-carnitine = O-octanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2, CRAT and 1 moreinner side of the membrane, outer side of the membraneOne of 3 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

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

• (5Z)-dodecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 2 moremitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 2 moremitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 2 moremitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ECHS1 and 2 moremitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ECHS1 and 2 moremitochondrial matrixOne of 2 routes

• CHEBI:27540Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• elaidic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (elaidate)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• 3beta-hydroxyandrost-5-en-17-oneReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. dehydroepiandrosterone 3-sulfate(out) + glutarate(in) = dehydroepiandrosterone 3-sulfate(in) + glutarate(out)
  3. 3beta-Hydroxyandrost-5-en-17-one 3-sulfate sulfohydrolase

  Catalysed by SUGCT, SLC22A24, SLC22A8, STSinner side of the membrane, outer side of the membrane, cytosolOne of 3 routes

• CHEBI:28701Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 2 moremitochondrial matrix, peroxisomeOne of 7 routes

• O-decanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2, CRATinner side of the membrane, outer side of the membrane

• a dicarboxylateReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. prostaglandin E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)
  3. prostaglandin E2(out) + a dicarboxylate(in) = prostaglandin E2(in) + a dicarboxylate(out)

  Catalysed by SUGCT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 8 routes

• pentanoateReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-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. itaconate + glutaryl-CoA = itaconyl-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. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCT and 2 moremitochondrial matrix

• (S)-lactic acidReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. transport of L-lactate and urate (cytosol to extracellular)

  Catalysed by SUGCT, SLC22A8, SLC22A12inner side of the membrane, outer side of the membrane, extracellular space, cytosol

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCTmitochondrial matrixOne of 4 routes

• taurineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, BAATmitochondrial matrix, cytosolOne of 2 routes

• O-propanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-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. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. acetoacetate + succinyl-CoA = acetoacetyl-CoA + succinate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCT and 1 moremitochondrial matrix

• glycineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 2 moremitochondrial matrixOne of 10 routes

• (R)-methylmalonyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  3. glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCT and 1 moremitochondrial matrixOne of 2 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• (2E)-glutaconyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCT and 1 moremitochondrial matrix

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 9 routes

• eicosanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 12 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 13 routes