3-methylmalate

Three steps

• N-decanoylglycineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

  Catalysed by CLYBL, AADAT, GLYATL2One of 2 routes

• N-octanoylglycineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

  Catalysed by CLYBL, AADAT, GLYATL2One of 2 routes

• octanamideReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. N-octanoyl-(2S)-hydroxyglycine = octanamide + glyoxylate
  3. N-octanoyl-(2S)-hydroxyglycine = octanamide + glyoxylate

  Catalysed by CLYBL, PAM

• (9Z,12Z,15Z)-octadecatrienamideReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycine = (9Z,12Z,15Z)-octadecatrienamide + glyoxylate
  3. N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycine = (9Z,12Z,15Z)-octadecatrienamide + glyoxylate

  Catalysed by CLYBL, PAM

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by CLYBL, AADAT, GART

• L-cystine-bis-glycineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by CLYBL, AADAT, DPEP1, DPEP2

• glycyldehydrophenylalanineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by CLYBL, AADAT, DPEP1

• an N-(fatty acyl)-glycineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by CLYBL, AADAT, GLYATL2

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by CLYBL, AADAT, GLYATL2

• acetylpyruvateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. acetylpyruvate + H2O = acetate + pyruvate + H(+)

  Catalysed by CLYBL, AGXT, AGXT2, FAHD1

• all-trans-retinoic acidReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. transport of pyruvate and retinoate (cytosol to extracellular)

  Catalysed by CLYBL, AGXT, AGXT2, SLC16A4 and 5 moreextracellular space, cytosolOne of 2 routes

• propionyl-CoAReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. 2-oxobutyrate oxidation

  Catalysed by CLYBL, AGXT2cytosolOne of 5 routes

• CHEBI:15570Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

• L-leucineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and leucine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A5extracellular space, cytosolOne of 7 routes

• CHEBI:15611Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by CLYBL, AADAT, GNMTcytosol

• CHEBI:15699Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and homoserine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A5extracellular space, cytosolOne of 4 routes

• formateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)
  3. oxalate(in) + formate(out) = oxalate(out) + formate(in)

  Catalysed by CLYBL, HAO1, SLC26A6inner side of the membrane, outer side of the membraneOne of 2 routes

• malonateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)

  Catalysed by CLYBL, SLC25A10inner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:15971Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and histidine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A6extracellular space, cytosolOne of 2 routes

• homogentisateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. 4-Hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by CLYBL, AADAT, HPDcytosolOne of 2 routes

• sulfateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)
  3. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by CLYBL, HAO1, SLC25A14, SLC26A2 and 3 moreinner side of the membrane, outer side of the membraneOne of 6 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by CLYBL, AGXT, AGXT2mitochondrial matrix

• (R)-carnitineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. propanoyl-CoA + (R)-carnitine = O-propanoyl-(R)-carnitine + CoA
  3. O-propanoyl-(R)-carnitine(in) + (R)-carnitine(out) = O-propanoyl-(R)-carnitine(out) + (R)-carnitine(in)

  Catalysed by CLYBL, CRAT, SLC25A20inner side of the membrane, outer side of the membraneOne of 2 routes

• L-citrullineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of citrulline and glycine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A9, SLC3A1 and 1 moreextracellular space, cytosol

• L-valineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and valine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A5extracellular space, cytosolOne of 4 routes

• oxaloacetateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. transport of malate and OAA (cytosol to mitochondria)

  Catalysed by CLYBLmitochondrial matrix, cytosolOne of 9 routes

• L-arginineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of arginine and glycine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A6extracellular space, cytosolOne of 2 routes

• CHEBI:16523Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

• protein polypeptide chainReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. PROTEIN_BS

  Catalysed by CLYBL, AADATcytosol

• (S)-lactateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by CLYBL, AGXT, AGXT2, LDHA and 4 more

• 2-oxoglutaramateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by CLYBL, AGXT, AGXT2, KYAT1cytosolOne of 3 routes

• enol-phenylpyruvateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  3. 3-phenylpyruvate = enol-phenylpyruvate

  Catalysed by CLYBL, AADAT, MIF

• 3-fumarylpyruvateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by CLYBL, AGXT, AGXT2, FAHD1

• CHEBI:16856Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by CLYBL, AADAT, GSScytosol

• CHEBI:16857Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and threonine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC3A2, SLC7A5 and 1 moreextracellular space, cytosolOne of 4 routes

• glyoxylic acidReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycolate + NADP(+) = glyoxylate + NADPH + H(+)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by CLYBL, GRHPRcytosolOne of 4 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. transport of (R)-3-hydroxybutanoate and pyruvate (cytosol to extracellular)

  Catalysed by CLYBL, AGXT, AGXT2, SLC16A4 and 5 moreextracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by CLYBL, AGXT, AGXT2

• L-isoleucineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and isoleucine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A5extracellular space, cytosolOne of 4 routes

• CHEBI:17203Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and proline (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A5, SLC7A6extracellular space, cytosolOne of 2 routes

• (S)-4-hydroxymandelateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by CLYBL, AADAT, HPDL

• aceneuramateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by CLYBL, AGXT, AGXT2, NPL

• 5'-deoxyadenosineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by CLYBL, AGXT, AGXT2, TYW1B and 1 more

• sulfiteReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. transport of malate and sulfite (cytosol to mitochondria)

  Catalysed by CLYBL, SLC25A10mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:17380Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O
  3. Kynurenine-Oxoglutarate Transaminase

  Catalysed by CLYBL, KYAT3, AADATmitochondrial matrixOne of 2 routes

• enol-oxaloacetateReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by CLYBL, SDHB, SDHA

• 5-aminolevulinic acidReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by CLYBL, AADAT, ALAS1, ALAS2 and 1 moremitochondrial matrix

• L-cysteineReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of cysteine and glycine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC3A2, SLC7A5 and 1 moreextracellular space, cytosolOne of 4 routes

• CHEBI:17895Reach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. transport of glycine and tyrosine (cytosol to extracellular)

  Catalysed by CLYBL, AADAT, SLC7A5extracellular space, cytosolOne of 4 routes

• fumaric acidReach from here →
  1. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by CLYBL, FHcytosolOne of 5 routes