(2S)-2-aminobutanoate

Three steps

• ureaReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by AGXT2, SLC7A6, ARG1extracellular space, cytosolOne of 3 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by AGXT2mitochondrial matrixOne of 3 routes

• 11-cis-retinolReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by AGXT2, BAAT, DGAT1, LRAT and 3 moreperoxisome, cytosol

• cob(II)alaminReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 2 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

  Catalysed by AGXT2, MOCOS, MTRR

• lithocholic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and lithocholate (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by AGXT2, SLC6A9, SLC6A14, SLC26A4extracellular space, cytosolOne of 25 routes

• oxaloacetateReach 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
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by AGXT2One of 10 routes

• nitric oxideReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by AGXT2, SLC7A6, NOS3, NOS1 and 1 moreextracellular space, cytosolOne of 3 routes

• selaneReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AGXT2, SCLYcytosolOne of 3 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-Arginine:glycine amidinotransferase
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by AGXT2, GATM, ACY1cytosol

• 2-phenylacetamideReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by AGXT2, SLC7A5, EPXextracellular space, cytosol

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

  Catalysed by AGXT2, KYNU

• D-alanyl-D-alanineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of D-alanine and glycine (cytosol to extracellular)
  3. Formation of Alanylalanine

  Catalysed by AGXT2, SLC3A2, SLC7A10extracellular space, cytosol

• Met-tRNA(Met)Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by AGXT2, SLC7A5, MARS1extracellular space, cytosol

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

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 2 more

• 3'-deoxy-3',4'-didehydro-CTPReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by AGXT2, MOCOS, RSAD2

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

  Catalysed by AGXT2, SCLY, PHOSPHO2, PDXPcytosolOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by AGXT2, AADATOne of 2 routes

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by AGXT2, SLC7A5, CNDP2extracellular space, cytosol

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

  Catalysed by AGXT2, SLC7A5, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by AGXT2, KYAT1cytosolOne of 5 routes

• (S)-2-hydroxyglutarateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by AGXT2, MOCOS, L2HGDH

• formaldehydeReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. sarcosine:oxygen oxidoreductase (demethylating)

  Catalysed by AGXT2, GNMT, PIPOX, SARDHcytosol, peroxisomeOne of 2 routes

• 3-fumarylpyruvateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1

• trans-1,2-dithiane-4,5-diolReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by AGXT2, SCLYcytosol

• creatineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by AGXT2, GATM, GAMTcytosolOne of 2 routes

• N-acetyl-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
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by AGXT2, NAT8Lmitochondrial matrixOne of 2 routes

• bilirubin IXalphaReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of bilirubin and GSH (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• oxalic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glycine:oxygen oxidoreductase (deaminating)
  3. Glyoxylate:oxygen oxidoreductase

  Catalysed by AGXT2, DAO, HAO1peroxisomeOne of 3 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of (R)-3-hydroxybutanoate (cytosol to extracellular)

  Catalysed by AGXT2, LDHB, LDHC, LDHAL6A and 7 morecytosol, extracellular spaceOne of 4 routes

• taurolithocholateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and taurolithocholate (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by AGXT2, AGXTOne of 2 routes

• CHEBI:17208Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by AGXT2, GSScytosol

• homocysteineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Formation of Homocystine

  Catalysed by AGXT2, GSScytosol

• L-glutamic 5-semialdehydeReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-Arginine:glycine amidinotransferase
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by AGXT2, GATM, OATcytosol, mitochondrial matrix

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

  Catalysed by AGXT2, BAAT, NAT2, NAT1peroxisome, cytosol

• aceneuramateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by AGXT2, NPL

• CHEBI:17312Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by AGXT2, BAATperoxisome, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + 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 AGXT2, TYW1B, TYW1One of 5 routes

• sulfiteReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by AGXT2, GSS, TSTD1One of 2 routes

• CHEBI:17380Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by AGXT2, SLC7A5extracellular space, cytosolOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by AGXT2, SLC7A5, SLC7A6, PRODH2extracellular space, cytosolOne of 3 routes

• agmatineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by AGXT2, SLC7A6extracellular space, cytosol, mitochondrial matrix

• indole-3-pyruvateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-tryptophan + glyoxylate = indole-3-pyruvate + glycine

  Catalysed by AGXT2, AADATOne of 3 routes

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by AGXT2, MOCOS, DIO1

• 3,3'-diiodo-L-thyronineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3One of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by AGXT2, MOCOS, DIO1One of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by AGXT2, BAAT, HADHA, ACADVL and 1 moreperoxisome, mitochondrial matrix

• CHEBI:17724Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by AGXT2, GNMT, PDPR, DMGDHcytosol

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

  Catalysed by AGXT2, SLC6A9, SLC6A14, SLC6A12extracellular space, cytosolOne of 3 routes