L-2-amino-3-oxobutanoic acid

Three steps

• nonadecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Activation of dodecanedioic acid

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of Sebacoylcarnitine

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of Suberylcarnitine

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

• 20-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Resistance-Nodulation-Cell Division (Rnd) Tcdb:2.A.60.1.2

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by GCAT, BAAT, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by GCAT, BAAT, ACAA1, HSD17B4 and 2 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of Hexadecanedioylcarnitine

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. fatty-acid-CoA ligase

  Catalysed by GCAT, BAAT, SLC27A2mitochondrial matrix, peroxisome, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by GCAT, BAAT, ACSM4, ACSM1 and 4 moremitochondrial matrix, peroxisome, cytosolOne of 3 routes

• a fatty acyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by GCAT, GLYATL2mitochondrial matrix

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (9S)-hydroperoxy-(10E,12Z)-octadecadienoate + 2 glutathione = (9S)-hydroxy-(10E,12Z)-octadecadienoate + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, GPX1, GPX4mitochondrial matrix

• ursodeoxycholateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and ursodeoxycholate (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin G2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin G2 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• thio-Mo-molybdopterinReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, GPX1, GPX2 and 1 moremitochondrial matrix

• (5Z)-tetradecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by GCAT, BAAT, ACADM, ECHS1 and 2 moremitochondrial matrix, peroxisome

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by GCAT, BAAT, HADHA, ACAA2 and 1 moremitochondrial matrix, peroxisome

• an N(2)-acyl-L-glutamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by GCAT, SLC7A5, GLYATL1B, GLYATL1mitochondrial matrix, extracellular space, cytosol

• (7Z)-hexadecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 8 routes

• 3-methylmalateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)

  Catalysed by GCAT, AADAT, CLYBLmitochondrial matrix

• tricosanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 4 routes

• (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, PTGES, MGST3 and 4 moremitochondrial matrix

• (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, GPX1, GPX4mitochondrial matrix

• thromboxane B2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and thromboxane B2 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO1B1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• an S-substituted glutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by GCAT, GSS, GSTT4, GSTA4 and 21 moremitochondrial matrixOne of 2 routes

• (13S)-hydroxy-(9Z,11E)-octadecadienoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (13S)-hydroperoxy-(9Z,11E)-octadecadienoate + 2 glutathione = (13S)-hydroxy-(9Z,11E)-octadecadienoate + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, GPX1, GSTA1 and 2 moremitochondrial matrix

• taurochenodeoxycholateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (GSH)

  Catalysed by GCAT, GSS, SLCO1A2, SLC7A9 and 1 moremitochondrial matrix, cytosol, extracellular spaceOne of 3 routes