4-methyl-2-oxopentanoate

Three steps

• (2S)-2-aminobutanoateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by AADAT, SLC7A5, AGXT2extracellular space, cytosolOne of 8 routes

• nonadecanoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by AADAT, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by AADAT, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Activation of dodecanedioic acid

  Catalysed by AADATmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Production of Sebacoylcarnitine

  Catalysed by AADAT, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Production of Suberylcarnitine

  Catalysed by AADAT, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by AADATmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by AADAT, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

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

• 2-iminobutanoateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. 2-iminobutanoate + H2O = 2-oxobutanoate + NH4(+)

  Catalysed by AADAT, AGXT2, RIDA

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by AADAT, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

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

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by AADAT, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by AADAT, ACAA1, HSD17B4, EHHADH and 1 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Production of Hexadecanedioylcarnitine

  Catalysed by AADAT, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. fatty-acid-CoA ligase

  Catalysed by AADAT, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by AADAT, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 3 routes

• 3-phenyllactateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  3. Formation of Phenyl-Lactate

  Catalysed by AADATcytosol

• thio-Mo-molybdopterinReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of cysteine and leucine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by AADAT, SLC7A9, SLC3A1, MOCOSextracellular space, cytosol

• 17beta-estradiol 17-O-(beta-D-glucuronate)Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. 17beta-estradiol 17-O-(beta-D-glucuronate)(out) + glutarate(in) = 17beta-estradiol 17-O-(beta-D-glucuronate)(in) + glutarate(out)

  Catalysed by AADAT, SLC22A6, SLC22A24inner side of the membrane, outer side of the membraneOne of 2 routes

• glycodeoxycholateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. Glycodeoxycholate amidohydrolase

  Catalysed by AADAT, SLC7A5extracellular space, cytosolOne of 2 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by AADAT, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by AADAT, ACADM, ECHS1, ACAA2 and 1 moremitochondrial matrix

• N-tetradecanoylglycineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by AADAT, SLC7A5, GLYATL2extracellular space, cytosolOne of 2 routes

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by AADAT, HADHA, ACAA2, HADHBmitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by AADAT, SLC7A5, BAATextracellular space, cytosol

• N-docosanoylglycineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by AADAT, SLC7A5, BAATextracellular space, cytosol

• N-hexacosanoylglycineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by AADAT, SLC7A5, BAATextracellular space, cytosol

• an N(2)-acyl-L-glutamineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glutamine and leucine (cytosol to extracellular)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by AADAT, SLC7A5, GLYATL1B, GLYATL1extracellular space, cytosol

• (7Z)-hexadecenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by AADAT, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by AADAT, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• 2-(1-hydroxyethyl)thiamine diphosphateReach from here →
  1. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)
  2. lipoamide to S-(3-methylbutanoyl)... conversion
  3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

  Catalysed by BCKDHA, BCKDHB, DBT, PDHA1 and 2 moremitochondrial matrixOne of 2 routes