(2S)-2-aminobutanoate

Two steps

• ophthalmateReach from here →
  1. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)
  2. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by GCLC, GSSOne of 2 routes

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by AGXT2, BCKDHA, BCKDHBmitochondrial matrix

• 2-aminoisobutanoateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate

  Catalysed by AGXT2One of 7 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by AGXT2One of 2 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by AGXT2, SHMT1cytosolOne of 2 routes

• CHEBI:240943Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by AGXT2, GLYAT, GLYATL2, GLYATL1mitochondrial matrix

• phenylacetylglycineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by AGXT2, GLYATcytosolOne of 3 routes

• selenomethionineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by AGXT2, CTHcytosol

• CHEBI:27760Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by AGXT2, CTHcytosol

• CHEBI:28666Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. LTD4 hydrolysis

  Catalysed by AGXT2cytosol

• pyridoxine 5'-phosphateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AGXT2, SCLYcytosol

• Gly-tRNA(Gly)Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by AGXT2, GARS1cytosol

• chlorine(.)Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of chloride and glycine (cytosol to extracellular)

  Catalysed by AGXT2, SLC6A9, SLC6A14extracellular space, cytosolOne of 5 routes

• glycocholateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by AGXT2, BAATperoxisomeOne of 3 routes

• L-aspartateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by AGXT2One of 2 routes

• L-lysineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-lysine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-lysine(in)

  Catalysed by AGXT2, SLC25A2inner side of the membrane, outer side of the membraneOne of 2 routes

• D-cysteineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 6 routes

• L-alpha-aminobutyric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by AGXT2, GPTcytosol

• 5-aminolevulinateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by AGXT2, ALAS1, ALAS2

• glycochenodeoxycholateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by AGXT2, BAAT

• glycochenodeoxycholic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by AGXT2, BAATperoxisomeOne of 3 routes

• glyoxylateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by AGXT2One of 12 routes

• glycolithocholic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by AGXT2, BAATcytosolOne of 2 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by AGXT2, GCATmitochondrial matrix

• L-ornithineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-Arginine:glycine amidinotransferase

  Catalysed by AGXT2, GATMcytosolOne of 10 routes

• gamma-Glu-AlaReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. gamma-glutamyltransferase (alanine)

  Catalysed by AGXT2, GGT1, GGT5, GGTLC2 and 3 moreextracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by AGXT2, GLDC, GCSHmitochondrial matrix

• D-alanineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by AGXT2, PIPOXOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by AGXT2, SHMT1, SHMT2

• an N-acylglycineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by AGXT2, GLYATL3, GLYAT, GLYATL2

• guanidinoacetateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-Arginine:glycine amidinotransferase

  Catalysed by AGXT2, GATMcytosolOne of 2 routes

• D-threonineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• L-methionineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and methionine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5extracellular space, cytosolOne of 12 routes

• L-tryptophanReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and tryptophan (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5extracellular space, cytosolOne of 9 routes

• glutathioneReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by AGXT2, GSS

• L-threonineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-threonine = 2-oxobutanoate + NH4(+)

  Catalysed by AGXT2, SDS, SDSLOne of 5 routes

• L-kynurenineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by AGXT2, KYNU

• beta-alanineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• leukotriene C4Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. leukotriene C4 hydrolysis

  Catalysed by AGXT2cytosol

• dimethylamineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by AGXT2, DDAH1cytosolOne of 2 routes

• L-asparagineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of asparagine and glycine (cytosol to extracellular)

  Catalysed by AGXT2extracellular space, cytosolOne of 10 routes

• L-phenylalanineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and phenylalanine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5extracellular space, cytosolOne of 13 routes

• 3-hydroxy-L-kynurenineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by AGXT2, KYNU

• L-tyrosineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by AGXT2, AADAT

• trans-4-hydroxy-L-prolineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by AGXT2, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

• L-2-aminopentanoateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by AGXT2

• kynurenateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O

  Catalysed by AGXT2, AADAT