itaconyl-CoA

Three steps

• an N-acylsphing-4-enineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)

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

• N-tetracosanoylsphinganineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

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

• N-hexacosanoylsphinganineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

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

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 6 moremitochondrial matrix, Golgi apparatus

• a 3-acylpyruvateReach from here →
  1. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)
  2. (3S)-citramalyl-CoA pyruvate-lyase (acetyl-CoA-forming)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by AUH, CLYBL, FAHD1mitochondrial matrix

• (3S)-3-hydroxybutanoyl-CoAReach from here →
  1. itaconate + succinyl-CoA = itaconyl-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. itaconate + succinyl-CoA = itaconyl-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

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

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

• 3-oxohexadecanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

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

• acryloyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Propanoate:CoA ligase (AMP-forming)
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSS3 and 4 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

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

• hexadecanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Carnitine O-Palmitoyltransferase
  3. (2E)-hexadecenoyl-CoA + NADPH + H(+) = hexadecanoyl-CoA + NADP(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 3 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

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

• propanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. (S)-methylmalonyl-CoA hydrolase
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ECHDC1mitochondrial matrix

• prostaglandin F2alphaReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  3. prostaglandin F2alpha(out) + glutarate(in) = prostaglandin F2alpha(in) + glutarate(out)

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

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

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

• sarcosineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

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

• S-adenosyl 3-(methylsulfanyl)propylamineReach 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, cytosol

• 7,8-dihydrofolateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

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

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

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

• propane-1,3-diamineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SAT1 and 2 moremitochondrial matrix, cytosol

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glycerol-3-phosphate 1-O-acyltransferase (palmitoyl-CoA)

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

• a 1-acylglycerone 3-phosphateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ASAH1 and 4 moremitochondrial matrix, Golgi apparatus

• O-acetyl-(R)-carnitineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Carnitine O-Palmitoyltransferase
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 2 moremitochondrial matrix, cytosolOne of 9 routes

• a 1,2-diacyl-sn-glycero-3-phosphocholineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acyl-sn-glycero-3-phosphocholine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphocholine + CoA

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

• an N-acylglycineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

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

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:2-oxoglutarate aminotransferase

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

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

  Catalysed by AUH, CLYBL, AGXT2mitochondrial matrix

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

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

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

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

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

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

• dehydroepiandrosterone 3-sulfateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  3. dehydroepiandrosterone 3-sulfate(out) + glutarate(in) = dehydroepiandrosterone 3-sulfate(in) + glutarate(out)

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

• L-tryptophanReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and tryptophan (cytosol to extracellular)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 3 moremitochondrial matrix, extracellular space, cytosolOne of 20 routes

• glutathioneReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

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

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-kynurenineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, KYNUmitochondrial matrix

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

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 3 routes

• a 1-acyl-sn-glycero-3-phosphateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sn-glycerol 3-phosphate + an acyl-CoA = a 1-acyl-sn-glycero-3-phosphate + CoA

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

• leukotriene C4Reach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. leukotriene C4 hydrolysis

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 2 moremitochondrial matrix, cytosol

• taurocyamineReach 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, cytosol

• L-cysteateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by SUCLG1, SUCLG2, SUCLA2, BAAT and 2 moremitochondrial matrix, cytosol

• L-phenylalanineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and phenylalanine (cytosol to extracellular)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, GLYAT and 3 moremitochondrial matrix, extracellular space, cytosolOne of 28 routes

• N(1)-acetylspermineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SAT1 and 2 moremitochondrial matrix, cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, KYNUmitochondrial matrix

• porphobilinogenReach from here →
  1. itaconate + succinyl-CoA = itaconyl-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

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

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

• L-homocysteineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. L-homocysteine + L-serine = L,L-cystathionine + H2O

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

• S-methyl-L-methionineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

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

• 3-oxosphinganineReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

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

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

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