3-methyl-(2E)-glutaconyl-CoA

Three steps

• L-cystine-bis-glycineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by GLYAT, GLYATL2, GLYATL1, DPEP1 and 1 moremitochondrial matrix

• an N-octadecanoyl-sphingoid baseReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a sphingoid base + octadecanoyl-CoA = an N-octadecanoyl-sphingoid base + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• glycyldehydrophenylalanineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by GLYAT, GLYATL2, GLYATL1, DPEP1mitochondrial matrix

• an N-(fatty acyl)-glycineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrix

• 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosolOne of 2 routes

• cholesteryl (7Z)-octadecenoateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. (7Z)-octadecenoyl-CoA + cholesterol = cholesteryl (7Z)-octadecenoate + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, SOAT1mitochondrial matrix, cytosol

• acetoneReach from here →
  1. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)
  2. hydroxymethylglutaryl-CoA lyase
  3. Acetoacetate carboxy-lyase (acetone-forming)

  Catalysed by AUH, HMGCLmitochondrial matrix, peroxisome

• acetylcholineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. Acetyl-CoA:choline O-acetyltransferase
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by CHATmitochondrial matrix, nucleus, cytosolOne of 3 routes

• pyruvateReach from here →
  1. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)
  2. hydroxymethylglutaryl-CoA lyase
  3. transport of acetoacetate and pyruvate (cytosol to mitochondria)

  Catalysed by AUH, HMGCLmitochondrial matrix, peroxisome, cytosolOne of 24 routes

• all-trans-retinoic acidReach from here →
  1. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)
  2. hydroxymethylglutaryl-CoA lyase
  3. transport of acetoacetate and retinoate (cytosol to extracellular)

  Catalysed by AUH, HMGCL, SLC16A4, SLC16A5mitochondrial matrix, peroxisome, extracellular space, cytosolOne of 2 routes

• CHEBI:15570Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC3A2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• (S)-malateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. ATP citrate lyase (citrate)
  3. transport of malate and OAA (cytosol to mitochondria)

  Catalysed by ACLYmitochondrial matrix, cytosolOne of 5 routes

• L-leucineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and leucine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 21 routes

• CHEBI:15611Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, GNMTmitochondrial matrix, cytosol

• CHEBI:15699Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and homoserine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 17 routes

• betaine aldehydeReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. Acetyl-CoA:choline O-acetyltransferase
  3. choline dehydrogenase (choline)

  Catalysed by CHAT, CHDHmitochondrial matrix, nucleus, cytosolOne of 3 routes

• trimethylenediamineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by SAT1, SAT2, DHPSmitochondrial matrix, cytosolOne of 2 routes

• hexadecanoic acidReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 4 routes

• 1-acylglycerone 3-phosphateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:glycerone-phosphate O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moremitochondrial matrix, Golgi apparatus, cytosol

• CHEBI:15971Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and histidine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 11 routes

• phosphatidylethanolamineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moremitochondrial matrix, Golgi apparatus, cytosol

• 11-cis-retinalReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol:NAD+ oxidoreductase

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moremitochondrial matrix, Golgi apparatus, cytosol

• hexadecan-1-olReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. Palmitate:CoA ligase (AMP-forming)
  3. formate dehydrogenase (palmitoyl-CoA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol, peroxisome

• 11-cis-retinyl hexadecanoateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 9 moremitochondrial matrix, cytosolOne of 3 routes

• L-cystineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by SLC7A9, SLC3A1mitochondrial matrix, extracellular space, cytosolOne of 5 routes

• (R)-3-aminoisobutyric acidReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by AGXT2mitochondrial matrix

• phosphatidic acidReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1-acyl-sn-glycerol-3-phosphate 2-O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 moremitochondrial matrix, Golgi apparatusOne of 7 routes

• L-citrullineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of citrulline and glycine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A9 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 8 routes

• L-valineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and valine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 20 routes

• L-arginineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of arginine and glycine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 18 routes

• CHEBI:16523Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC3A2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• protein polypeptide chainReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. PROTEIN_BS

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrix, cytosolOne of 5 routes

• CHEBI:16633Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SCLYmitochondrial matrix, cytosol

• Met-tRNA(Met)Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by NAGS, MARS1mitochondrial matrix, cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by NAGS, AADATmitochondrial matrix

• 2-oxobutanoateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. L-cystathionine cysteine-lyase (deaminating

  Catalysed by NAGS, CTHmitochondrial matrix, cytosolOne of 2 routes

• CHEBI:16856Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. N-acetyl-LTE4 to leukotriene E4 conversion
  3. transport of GSH and leukotriene E4 (cytosol to extracellular)

  Catalysed by ZDHHC11B, NAA10, ESCO2, ESCO1 and 29 moremitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• CHEBI:16857Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and threonine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC3A2 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 19 routes

• pantetheine 4'-phosphateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acid phosphatase 2, lysosomal (CoA)
  3. Dephospho-CoA nucleotidohydrolase

  Catalysed by ACP2, ENPP3, ENPP1mitochondrial matrix, lysosome, cytosolOne of 2 routes

• psychosineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2mitochondrial matrix, Golgi apparatus, cytosol

• glyoxylic acidReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by GLYAT, GLYATL2, GLYATL1, DAOmitochondrial matrix, peroxisome

• 1-acyl-sn-glycerol 3-phosphateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moremitochondrial matrix, Golgi apparatus

• a cholesterol esterReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + cholesterol = a cholesterol ester + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moremitochondrial matrix, Golgi apparatus

• (R)-3-hydroxybutyric acidReach from here →
  1. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)
  2. hydroxymethylglutaryl-CoA lyase
  3. (R)-3-Hydroxybutanoate:NAD+ oxidoreductase

  Catalysed by AUH, HMGCL, BDH1, BDH2mitochondrial matrix, peroxisomeOne of 4 routes

• taurolithocholateReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. bile acid-CoA:amino acid N-acyltransferase (lithocholate)

  Catalysed by BAATmitochondrial matrix, cytosolOne of 2 routes

• L-isoleucineReach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and isoleucine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 18 routes

• CHEBI:17203Reach from here →
  1. Formation of 3-Methyl-Glutaconate
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and proline (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 15 routes

• succinyl-4'-phosphopantetheineReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = 3-methyl-(2E)-glutaconyl-CoA + H2O
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. succinyl-CoA + H2O = succinyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by AUH, SUGCT, NUDT19, NUDT7 and 1 more