CHEBI:15345

Two steps

• glutaryl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Production of Glutaryl Carnitine

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1BcytosolOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Itaconate:CoA ligase (GDP-forming)

  Catalysed by HMGCS1, SUCLG1, SUCLG2, SUCLA2cytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by HMGCS1, CROTcytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. phytanate-CoA ligase

  Catalysed by HMGCS1cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 4 morecytosolOne of 15 routes

• sphing-4-enineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by HMGCS1, ASAH1, ACER2, ACER1 and 1 morecytosol, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by HMGCS1, SAT1, SAT2cytosolOne of 3 routes

• L-methionineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetylglutamate synthase (N-acetylmethionine)

  Catalysed by HMGCS1, NAGScytosol, mitochondrial matrix

• L-alanineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)

  Catalysed by HMGCS1cytosol, mitochondrial matrix

• L-asparagineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetylglutamate synthase (N-acetyl-L-asparagine)

  Catalysed by HMGCS1, NAGScytosol, mitochondrial matrix

• L,L-cystathionineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by HMGCS1, NAGScytosol, mitochondrial matrix

• an acyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by HMGCS1, ASAH1, ACER2, ACER1 and 1 morecytosol, Golgi apparatusOne of 2 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by HMGCS1, HADHA, ACAA2, HADHBcytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by HMGCS1, SLC27A2, SLC27A5cytosol, endoplasmic reticulumOne of 3 routes

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

  Catalysed by HMGCS1, SLC27A2, SLC27A5cytosolOne of 4 routes

• decanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by HMGCS1, ACSM4, ACSM1, ACSM2A and 3 morecytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 11 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by HMGCS1, CPT1A, CPT1Bcytosol

• CHEBI:63544Reach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 9 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1BcytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 8 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 9 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 4 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1BcytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Activation of dodecanedioic acid

  Catalysed by HMGCS1cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Production of Sebacoylcarnitine

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Production of Suberylcarnitine

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by HMGCS1cytosolOne of 4 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes