(R)-3-aminoisobutyric acid

Three steps

• N-benzoylglycineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by AGXT2, AGXTmitochondrial matrixOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by AGXT2, SLC7A5, CDO1mitochondrial matrix, extracellular space, cytosolOne of 6 routes

• decanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by AGXT2, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by ABAT, AGXT2, HOGA1mitochondrial matrix

• 3-hydroxyisovaleryl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by AGXT2, CPT1A, CPT1Bmitochondrial matrix, cytosol

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

  Catalysed by AGXT2, SLC1A4, PYCR1, PYCR3 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• CHEBI:63544Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• a 2,3-saturated acyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, ACAD10mitochondrial matrix

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by AGXT2, SLC7A6, NOS3, NOS1 and 1 moremitochondrial matrix, extracellular space, cytosol

• xanthurenateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by AGXT2, KYNU, KYAT3, AADATmitochondrial matrix

• (2E)-octadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by ABAT, AGXT2, HOGA1mitochondrial matrix

• L-threonylcarbamoyladenylateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

  Catalysed by AGXT2, SLC7A10, YRDCmitochondrial matrix, inner side of the membrane, outer side of the membrane

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Activation of dodecanedioic acid

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Sebacoylcarnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Suberylcarnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• 2-aminoprop-2-enoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-serine = 2-aminoprop-2-enoate + H2O

  Catalysed by AGXT2, SLC7A10, SRRmitochondrial matrix, inner side of the membrane, outer side of the membrane

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by AGXT2, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by AGXT2, ACAA1, HSD17B4, EHHADH and 1 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Hexadecanedioylcarnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. fatty-acid-CoA ligase

  Catalysed by AGXT2, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by AGXT2, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 3 routes