(2S)-2-aminobutanoate

Three steps

• pyridoxal 5'-phosphateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by AGXT2, SCLY, PNPOcytosol

• an L-alpha-amino acidReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

  Catalysed by AGXT2, KYNU, SLC7A11inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of chloride and glycine (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by AGXT2, SLC6A9, SLC6A14, SLC6A12extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

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

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

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

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

  Catalysed by AGXT2, BAAT, SULT2A1cytosolOne of 3 routes

• prostaglandin E2Reach 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 E2 (cytosol to extracellular)

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

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

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosolOne of 6 routes

• decanoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by AGXT2, BAAT, ACSM4, ACSM1 and 4 moreperoxisome, cytosolOne of 8 routes

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

  Catalysed by AGXT2, BAAT, CPT1A, CPT1C and 1 moreperoxisome, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-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 (palmitolate)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 11 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by AGXT2, HOGA1One of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by AGXT2, BAAT, CPT1A, CPT1Bperoxisome, cytosol

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Transport of Hydroxy-Proline
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by AGXT2, SLC1A4, PYCR1, PYCR3 and 1 moreextracellular space, cytosolOne of 5 routes

• leukotriene D4Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. leukotriene C4 hydrolysis
  3. leukotriene C4 + H2O = leukotriene D4 + L-glutamate

  Catalysed by AGXT2, GGT1, GGT5cytosol

• CHEBI:63544Reach 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 (adrenic acid)

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

• ubiquinol-10Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. ubiquinone-10 + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + ubiquinol-10

  Catalysed by AGXT2, GSS, SQOR

• hexacosanoyl-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 (cerotic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• tetracosanoyl-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 (lignocerate)

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

• docosanoyl-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 (behenic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 9 routes

• a 2,3-saturated acyl-CoAReach 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. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by AGXT2, MOCOS, ACAD10

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach 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 (N-(omega)-hydroxyarginine-forming)

  Catalysed by AGXT2, SLC7A6, NOS3, NOS1 and 1 moreextracellular space, cytosol

• xanthurenateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by AGXT2, KYAT3, AADATOne of 2 routes

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

  Catalysed by AGXT2, BAAT, CPT1A, CPT1C and 1 moreperoxisome, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-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 (stearidonic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 8 routes

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by AGXT2, HOGA1One of 2 routes

• (R)-noradrenalineReach 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 noradrenaline (cytosol to extracellular)

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

• L-threonylcarbamoyladenylateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-threonine = 2-oxobutanoate + NH4(+)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

  Catalysed by AGXT2, SDS, SDSL, YRDC

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-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 (EPA)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-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 (DPA)

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

• (9Z,12Z,15Z)-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 (linolenate)

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

• (13Z)-docosenoyl-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 (cis-erucic acid)

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

• (11Z)-eicosenoyl-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 (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-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 ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-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,12Z,15Z,18Z,21Z)-THA)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-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 (nervonic acid)

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

• (17Z)-hexacosenoyl-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 (ximenic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-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 (dihomo-gamma-linolenate)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-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 (omega-3-arachidonic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-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 (DHA)

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

• heptadecanoyl-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 (margaric acid)

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

• (9Z)-heptadecenoyl-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 (9-heptadecylenic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-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 ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• nonadecanoyl-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 (nonadecylic acid)

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

• (11Z)-octadecenoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by AGXT2, BAAT, CPT1A, CPT1C and 1 moreperoxisome, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Activation of dodecanedioic acid

  Catalysed by AGXT2, BAATperoxisome, cytosolOne of 4 routes

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

  Catalysed by AGXT2, BAAT, CPT1A, CPT1C and 1 moreperoxisome, cytosolOne of 4 routes

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

  Catalysed by AGXT2, BAAT, CPT1A, CPT1C and 1 moreperoxisome, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by AGXT2, BAATperoxisome, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-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 ((4Z,7Z,10Z,13Z,16Z)-DPA)

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