itaconyl-CoA

Three steps

• 2-aminoisobutanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 11-cis-retinyl esterReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, DGAT1 and 4 moremitochondrial matrix, cytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. ATP citrate lyase (citrate)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACLY and 1 moremitochondrial matrix, cytosolOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

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

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by AUH, CLYBL, AGXT2mitochondrial matrix

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by AUH, CLYBL, AGXT2mitochondrial matrix

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 7 moremitochondrial matrix, cytosol

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 9 moremitochondrial matrix, cytosol

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 3 moremitochondrial matrix, cytosolOne of 2 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 6 moremitochondrial matrix, cytosol

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

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

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 9 moremitochondrial matrix, cytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 10 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 4 moremitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 2 moremitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 4 moremitochondrial matrix

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ECHS1 and 3 moremitochondrial matrixOne of 3 routes

• docosanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 9 moremitochondrial matrix, cytosolOne of 4 routes

• CHEBI:240943Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

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

• octadecanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Stearoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 11 moremitochondrial matrix, cytosolOne of 4 routes

• octanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CROT and 1 moremitochondrial matrix, cytosolOne of 2 routes

• alpha-linolenic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 2 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 2 moremitochondrial matrix, cytosolOne of 6 routes

• decanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. Decanoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 9 moremitochondrial matrix, cytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 8 moremitochondrial matrix, cytosol

• CHEBI:28666Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetyl-LTE4 to leukotriene E4 conversion
  3. LTD4 hydrolysis

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ZDHHC11B and 31 moremitochondrial matrix, cytosolOne of 2 routes

• O-decanoyl-(R)-carnitineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 7 moremitochondrial matrix, cytosol

• pyridoxine 5'-phosphateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SCLYmitochondrial matrix, cytosol

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, NAGSmitochondrial matrix

• K(+)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, NAGS and 1 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• Cys-tRNA(Cys)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

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

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 3 moremitochondrial matrix, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-asparagine)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

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

• chlorine(.)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Carnitine O-Palmitoyltransferase
  3. Carnitine Transport by Atb0

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 3 moremitochondrial matrix, cytosol, extracellular spaceOne of 15 routes

• phosphocholineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CHAT and 1 moremitochondrial matrix, nucleusOne of 2 routes

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 3 moremitochondrial matrix, peroxisomeOne of 7 routes

• cholateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. propanoyl-CoA C-acyltransferase
  3. choloyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 3 moremitochondrial matrix, peroxisome, cytosolOne of 2 routes

• hydrogen sulfideReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by SUCLG1, SUCLG2, SUCLA2, NAGS and 1 moremitochondrial matrix, cytosolOne of 2 routes

• L-aspartateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, AGXT2mitochondrial matrixOne of 12 routes

• acetateReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. transport of acetate and pyruvate (cytosol to extracellular)

  Catalysed by AUH, CLYBL, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (9Z,12Z)-octadecadienoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 11 moremitochondrial matrix, cytosol

• 3-acetamidopropanalReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, PAOX and 1 moremitochondrial matrix, cytosolOne of 4 routes

• formic acidReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. transport of formate and pyruvate (cytosol to extracellular)

  Catalysed by AUH, CLYBL, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• propionic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Propanoate:CoA ligase (AMP-forming)
  3. Propanoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSS3 and 3 moremitochondrial matrix, cytosolOne of 5 routes

• citric acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. ATP citrate lyase (citrate)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACLY and 1 moremitochondrial matrix, cytosolOne of 2 routes

• butyric acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Butanoate:CoA ligase (AMP-forming)
  3. Butyryl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 6 moremitochondrial matrix, cytosolOne of 3 routes

• tetradecanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. Myristoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 12 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 2 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes