CHEBI:15345

Three steps

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

  Catalysed by HMGCS1, CROT, ACOT7cytosol, mitochondrial matrixOne of 2 routes

• alpha-linolenic acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1Bcytosol, endoplasmic reticulumOne of 2 routes

• phenylacetylglycineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by HMGCS1, GLYAT, GLYATL2, GLYATL1cytosol, mitochondrial matrixOne of 6 routes

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

  Catalysed by HMGCS1, ACSM4, ACSM1, ACSM2A and 4 morecytosolOne of 2 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 7 morecytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 6 morecytosol

• CHEBI:28666Reach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetyl-LTE4 to leukotriene E4 conversion
  3. LTD4 hydrolysis

  Catalysed by HMGCS1, ZDHHC11B, NAA10, ESCO2 and 29 morecytosolOne of 2 routes

• O-decanoyl-(R)-carnitineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by HMGCS1, ACSM4, ACSM1, ACSM2A and 5 morecytosol

• pyridoxine 5'-phosphateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by HMGCS1, SCLYcytosol, mitochondrial matrix

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

  Catalysed by HMGCS1, NAGScytosol, mitochondrial matrix

• K(+)Reach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  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 HMGCS1, NAGS, SLC1A1cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by HMGCS1, NAGS, CARS1cytosol, mitochondrial matrix

• Gly-tRNA(Gly)Reach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by HMGCS1, GLYAT, GLYATL2, GLYATL1 and 1 morecytosol, mitochondrial matrix

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

  Catalysed by HMGCS1, NAGS, NARS1cytosol, mitochondrial matrix

• chlorine(.)Reach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. Carnitine Transport by Atb0

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1B and 1 morecytosol, extracellular spaceOne of 15 routes

• phosphocholineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by HMGCS1, CHAT, PHOSPHO1cytosol, nucleusOne of 2 routes

• glycocholateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. propanoyl-CoA C-acyltransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by HMGCS1, ACAA1, SCP2, MYO5B and 1 morecytosol, peroxisomeOne of 5 routes

• cholateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. propanoyl-CoA C-acyltransferase
  3. choloyl-CoA hydrolase

  Catalysed by HMGCS1, ACAA1, SCP2, MYO5B and 1 morecytosol, peroxisomeOne of 2 routes

• hydrogen sulfideReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by HMGCS1, NAGS, CTHcytosol, mitochondrial matrixOne of 2 routes

• L-aspartateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by HMGCS1, AGXT2cytosol, mitochondrial matrixOne of 12 routes

• acetateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. hydroxymethylglutaryl-CoA lyase
  3. transport of acetate and acetoacetate (cytosol to extracellular)

  Catalysed by HMGCS1, HMGCL, SLC16A4, SLC16A5 and 3 morecytosol, peroxisome, extracellular spaceOne of 2 routes

• (9Z,12Z)-octadecadienoateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 9 morecytosol

• 3-acetamidopropanalReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by HMGCS1, PAOX, SMOXcytosolOne of 4 routes

• formic acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. hydroxymethylglutaryl-CoA lyase
  3. transport of acetoacetate and formate (cytosol to extracellular)

  Catalysed by HMGCS1, HMGCL, SLC16A4, SLC16A5cytosol, peroxisome, extracellular spaceOne of 2 routes

• propionic acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Propanoate:CoA ligase (AMP-forming)
  3. Propanoyl-CoA hydrolase

  Catalysed by HMGCS1, ACSS3, ACSS2, ACSS1 and 1 morecytosolOne of 5 routes

• citric acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. ATP citrate lyase (citrate)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by HMGCS1, ACLY, CScytosol, mitochondrial matrixOne of 2 routes

• butyric acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Butanoate:CoA ligase (AMP-forming)
  3. Butyryl-CoA hydrolase

  Catalysed by HMGCS1, ACSM4, ACSM1, ACSM2A and 4 morecytosolOne of 3 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 10 morecytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1Bcytosol, endoplasmic reticulumOne of 3 routes

• adipic acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by HMGCS1, ACOT8cytosol, peroxisomeOne of 3 routes

• glutarateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + H2O = glutarate + CoA + H(+)

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1B and 2 morecytosolOne of 6 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. ATP citrate lyase (citrate)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by HMGCS1, ACLY, GOT2cytosol, mitochondrial matrix

• docosan-1-olReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 6 morecytosol

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 7 morecytosolOne of 2 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 7 morecytosolOne of 4 routes

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

  Catalysed by HMGCS1, ASAH1, ACER2, ACER1 and 7 morecytosol, Golgi apparatus

• octadecan-1-olReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 6 morecytosol

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 8 morecytosolOne of 4 routes

• (9Z)-hexadecenoateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1Bcytosol, endoplasmic reticulumOne of 8 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 7 morecytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by HMGCS1, CPT1A, CPT1C, CPT1Bcytosol, endoplasmic reticulumOne of 4 routes

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

  Catalysed by HMGCS1, SLC27A2, ACSL4, ACSL3 and 8 morecytosol

• putrescineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by HMGCS1, SAT1, SAT2, SRMcytosolOne of 3 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by HMGCS1, NAGS, IL4I1cytosol, mitochondrial matrix

• di-mu-sulfido-diiron(2+)Reach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. De novo synthesis of 2Fe2S

  Catalysed by HMGCS1, NAGS, NDUFAB1, FDXR and 5 morecytosol, mitochondrial matrix

• D-cysteineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by HMGCS1, SLC7A10cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by HMGCS1, SLC7A10cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 6 routes

• an acylcholineReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by HMGCS1, CHAT, BCHEcytosol, nucleus

• fatty acidReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (elaidate)
  3. (10Z)-heptadecenoic acid formation

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

• 5-aminolevulinateReach from here →
  1. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by HMGCS1, GLYAT, GLYATL2, GLYATL1 and 2 morecytosol, mitochondrial matrix