itaconyl-CoA

Three steps

• adipic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 4 routes

• 3-hydroxypyruvic acidReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. L-Serine:pyruvate aminotransferase

  Catalysed by AUH, CLYBL, AGXTmitochondrial matrix, cytosolOne of 2 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. ATP citrate lyase (citrate)
  3. D-aspartate:2-oxoglutarate aminotransferase

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

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

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

• hexacosanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. Hexacosanoyl-CoA hydrolase

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

• tetracosanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. Tetracosenoyl-CoA hydrolase

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

• an N-acylsphinganineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphingosine N-acyltransferase (sphinganine)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 9 moremitochondrial matrix, Golgi apparatus, cytosol

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

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

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 10 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoateReach 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 8 routes

• (15Z)-tetracosenoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)
  3. (15Z)-tetracosenoyl-CoA hydrolase

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

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach 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 4 routes

• (11Z)-eicosenoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
  3. (11Z)-eicosenoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 10 moremitochondrial matrix, cytosol

• putrescineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SAT1 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• 3-oxopropanoateReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by AUH, CLYBL, AGXT2mitochondrial matrix

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

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

• di-mu-sulfido-diiron(2+)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. De novo synthesis of 2Fe2S

  Catalysed by SUCLG1, SUCLG2, SUCLA2, NAGS and 7 moremitochondrial matrix

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

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

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 7 routes

• an acylcholineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CHAT and 1 moremitochondrial matrix, nucleus

• fatty acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (elaidate)
  3. (10Z)-heptadecenoic acid formation

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• chenodeoxycholateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. chenodeoxycholoyl-CoA hydrolase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 5 moremitochondrial matrix, peroxisomeOne of 3 routes

• glycochenodeoxycholateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

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

• taurocholateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. propanoyl-CoA C-acyltransferase
  3. Choloyl-CoA:glycine N-choloyltransferase

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

• taurodeoxycholateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 2 moremitochondrial matrix, cytosolOne of 4 routes

• glycochenodeoxycholic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 3 moremitochondrial matrix, peroxisomeOne of 5 routes

• (R)-mevalonateReach from here →
  1. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. (R)-mevalonate + 2 NADP(+) + CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + 2 NADPH + 2 H(+)

  Catalysed by SUGCT, HMGCR

• cholesteryl hexadecanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Acyl-CoA:cholesterol O-acyltransferase

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

• glyoxylateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 3 moremitochondrial matrixOne of 11 routes

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 7 moremitochondrial matrix, Golgi apparatus, cytosol

• 3-hydroxyisovaleric acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  3. Formation of 3-Hydroxy-Isovalerate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 1 moremitochondrial matrix, cytosol

• 3,7,11,15-tetramethylhexadecanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. phytanate-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• sphingosine 1-phosphateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 6 moremitochondrial matrix, Golgi apparatus, cytosolOne of 2 routes

• glycolithocholic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. Acetyl-CoA:glycine C-acetyltransferase

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

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 9 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• (R)-lactic acidReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by AUH, CLYBL, LDHDmitochondrial matrix, cytosol

• (S)-lactic acidReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by AUH, CLYBL, LDHB, LDHAL6Bmitochondrial matrixOne of 5 routes

• 2-iminopropanoateReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by AUH, CLYBL, RIDAmitochondrial matrix

• phosphoenolpyruvic acidReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. ATP citrate lyase (citrate)
  3. GTP:oxaloacetate carboxy-lyase (adding GTP

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACLY and 1 moremitochondrial matrix, cytosolOne of 3 routes

• 13-cis-retinolReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 13-cis-retinol

  Catalysed by SUCLG1, SUCLG2, SUCLA2, DGAT1 and 5 moremitochondrial matrix, cytosol

• spermineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SAT1 and 2 moremitochondrial matrix, cytosolOne of 2 routes

• N-acetyl-beta-neuraminic acidReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by AUH, CLYBL, NPLmitochondrial matrix, cytosol

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 4 moremitochondrial matrix, cytosol, endoplasmic reticulum

• L-ornithineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by SUCLG1, SUCLG2, SUCLA2, BAAT and 1 moremitochondrial matrix, cytosolOne of 15 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 4 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• a 1,3-diacylglycerolReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 5 moremitochondrial matrix, Golgi apparatus

• a 1,2-diacylglycerolReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 8 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• gamma-Glu-AlaReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GGT1 and 5 moremitochondrial matrix, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

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