CHEBI:15380

Two steps

• itaconyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Itaconate:CoA ligase (GDP-forming)

  Catalysed by ALAS1, CPNE7, SUCLG1, SUCLG2 and 1 moremitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ALAS1, CPNE7, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. phytanate-CoA ligase

  Catalysed by ALAS1, CPNE7mitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 5 moremitochondrial matrix, cytosolOne of 15 routes

• sphing-4-enineReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ALAS1, CPNE7, ASAH1, ACER2 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by ALAS1, CPNE7, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

• L-methionineReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. N-acetylglutamate synthase (N-acetylmethionine)

  Catalysed by ALAS1, CPNE7, NAGSmitochondrial matrix

• L-alanineReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)

  Catalysed by ALAS1, CPNE7mitochondrial matrix

• L-asparagineReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. N-acetylglutamate synthase (N-acetyl-L-asparagine)

  Catalysed by ALAS1, CPNE7, NAGSmitochondrial matrix

• porphobilinogenReach from here →
  1. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  2. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by ALAS1, ALAS2, CPNE7, ALADmitochondrial matrix, cytosol

• L,L-cystathionineReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by ALAS1, CPNE7, NAGSmitochondrial matrix

• an acyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ALAS1, CPNE7, ASAH1, ACER2 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by ALAS1, CPNE7, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 3 routes

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ALAS1, CPNE7, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ALAS1, CPNE7, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• decanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ALAS1, CPNE7, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by ALAS1, CPNE7, CPT1A, CPT1Bmitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Activation of dodecanedioic acid

  Catalysed by ALAS1, CPNE7mitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Production of Sebacoylcarnitine

  Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Production of Suberylcarnitine

  Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by ALAS1, CPNE7mitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes