(2S)-2-aminobutanoate

Three steps

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by AGXT2, SLC7A5, BCAT2, BCAT1extracellular space, cytosol

• 2-oxohexanoateReach 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. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by AGXT2One of 2 routes

• 3-methyl-2-oxovaleric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and isoleucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, SLC7A5, BCAT1extracellular space, cytosolOne of 2 routes

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

  Catalysed by AGXT2, AADATOne of 7 routes

• taurocholateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (GSH)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

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

  Catalysed by AGXT2, KYNU

• (R)-lactic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by AGXT2, LDHDcytosol

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

  Catalysed by AGXT2, LDHB, LDHC, LDHAL6A and 8 morecytosol, extracellular spaceOne of 7 routes

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by AGXT2, MOCOS, CH25H

• 2-iminopropanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by AGXT2, RIDA

• phosphoenolpyruvic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. pyruvate kinase (dTDP)

  Catalysed by AGXT2, PKM, PKLRcytosol

• N-acetyl-beta-neuraminic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by AGXT2, NPLcytosol

• tert-butanolReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, CLIC2, GPX1 and 3 more

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

  Catalysed by AGXT2, BAATperoxisome, cytosolOne of 4 routes

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by AGXT2, MOCOS, RETSAT

• 3,3',5-triiodo-L-thyronineReach 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 triiodothyronine (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

• 3,3',5'-triiodo-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',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3

• a 3-acylpyruvateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1

• (R)-methylmalonyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by AGXT2, BAAT, ALDH6A1peroxisome, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutyrate oxidation
  3. propanoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by AGXT2, PCCA, PCCBcytosol, mitochondrial matrixOne of 2 routes

• 3'-dephospho-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by AGXT2, BAAT, ACP2peroxisome, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by AGXT2, BAATperoxisome, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by AGXT2, BAAT, ACAA1, MYO5BperoxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 7 routes

• acryloyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutyrate oxidation
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by AGXT2, ACADS, ACADSBcytosol, mitochondrial matrix

• benzoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by AGXT2, GLYATmitochondrial matrix

• butanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by AGXT2, BAAT, ACSM4, ACSM1 and 4 moreperoxisome, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 10 routes

• glutaryl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of Glutaryl Carnitine

  Catalysed by AGXT2, BAAT, CPT1A, CPT1C and 1 moreperoxisome, cytosolOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by AGXT2, GLYATL3

• eicosanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by AGXT2, BAAT, SUCLG1, SUCLG2 and 1 moreperoxisome, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 14 routes

• tetradecanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 12 routes

• octanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by AGXT2, BAAT, CROTperoxisome, mitochondrial matrixOne of 21 routes

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

  Catalysed by AGXT2, BAATperoxisome, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 5 moreperoxisome, cytosolOne of 15 routes

• prostaglandin E1Reach 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 prostaglandin E1 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach 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 prostaglandin I2 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach 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 prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach 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 prostaglandin H2 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 4 routes

• prostaglandin D2Reach 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 prostaglandin D2 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by AGXT2, GSS, GPX5, GPX1 and 7 morecytosolOne of 3 routes

• 7,8-dihydrofolateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase

  Catalysed by AGXT2, SHMT1, DHFR, DHFR2cytosolOne of 3 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by AGXT2, SHMT1, FTCDcytosolOne of 2 routes

• leukotriene A4Reach 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 leukotriene A4 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 3 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of arginine and glycine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by AGXT2, SLC7A6, ASLextracellular space, cytosolOne of 6 routes

• (R)-S-lactoylglutathioneReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by AGXT2, GSS, GLO1, GLOD4cytosolOne of 3 routes

• carnosineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by AGXT2, SLC7A10, CARNS1inner side of the membrane, outer side of the membrane

• 4-guanidinobutanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of arginine and glycine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by AGXT2, SLC7A6, GATMextracellular space, cytosol