(R)-3-aminoisobutyric acid

Three steps

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by AGXT2, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:15380Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  3. (R)-Methylmalonyl-CoA CoA-carbonylmutase

  Catalysed by ABAT, AGXT2, ALDH6A1, MMUTmitochondrial matrixOne of 9 routes

• CHEBI:15477Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by AGXT2, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

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

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

• isobutyryl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by AGXT2, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by AGXT2, GLYATmitochondrial matrix

• isovaleryl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by AGXT2, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 3 routes

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

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

• CHEBI:15519Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by AGXT2, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

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

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

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

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 11 routes

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

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 3 routes

• (S)-malateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-malate + NAD(+) = pyruvate + CO2 + NADH

  Catalysed by ABAT, AGXT2, ME2mitochondrial matrixOne of 2 routes

• CHEBI:15611Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by AGXT2, AGXT, GNMTmitochondrial matrix, cytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by AGXT2, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:15650Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by AGXT2, ZDHHC11B, NAA10, ESCO2 and 29 moremitochondrial matrix, cytosolOne of 2 routes

• trimethylenediamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by AGXT2, SLC7A6, DHPSmitochondrial matrix, extracellular space, cytosol

• CHEBI:15727Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by AGXT2, NAA10mitochondrial matrix, cytosolOne of 3 routes

• CHEBI:15746Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by AGXT2, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by AGXT2, SLC7A5, THmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by AGXT2, CADmitochondrial matrix, cytosol

• alpha-D-glucosamine 6-phosphateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A10 and 2 moremitochondrial matrix, extracellular space, cytosol

• thioredoxin dithiolReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:tRNA(Ala) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by AGXT2, AARS1mitochondrial matrix, cytosolOne of 2 routes

• sulfateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and chloride (cytosol to extracellular)
  3. transport of chloride and sulfate (cytosol to extracellular)

  Catalysed by AGXT2, SLC6A14, SLC26A6mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• ureaReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by AGXT2, SLC7A6, ARG1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• 11-cis-retinolReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by AGXT2, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosol

• cob(II)alaminReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, MTRRmitochondrial matrix

• iodideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by AGXT2, SLC7A5, TPOmitochondrial matrix, extracellular space, cytosolOne of 25 routes

• oxaloacetateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Pyruvate:carbon-dioxide ligase (ADP-forming)

  Catalysed by ABAT, AGXT2, PCmitochondrial matrixOne of 9 routes

• nitric oxideReach 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 (Nitric-Oxide-Forming)

  Catalysed by AGXT2, SLC7A6, NOS3, NOS1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• selaneReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AGXT2, SCLYmitochondrial matrix, cytosolOne of 3 routes

• CHEBI:16523Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by AGXT2, AGXT, SLC3A2, SLC7A10mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by AGXT2, SLC7A6, ACY1mitochondrial matrix, extracellular space, cytosol

• 2-phenylacetamideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by AGXT2, SLC7A5, EPXmitochondrial matrix, extracellular space, cytosol

• anthranilateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by AGXT2, KYNUmitochondrial matrix

• D-alanyl-D-alanineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. alanine racemase
  3. Formation of Alanylalanine

  Catalysed by AGXT2mitochondrial matrix, cytosol

• Met-tRNA(Met)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by AGXT2, SLC7A5, MARS1mitochondrial matrix, extracellular space, cytosol

• (S)-lactateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by ABAT, AGXT2, LDHA, LDHB and 3 moremitochondrial matrix

• 3'-deoxy-3',4'-didehydro-CTPReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, RSAD2mitochondrial matrix

• pyridoxineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine-5'-phosphate phosphohydrolase

  Catalysed by AGXT2, SCLY, PHOSPHO2, PDXPmitochondrial matrix, cytosolOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by AGXT2, SLC7A5, AADATmitochondrial matrix, extracellular space, cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by AGXT2, SLC7A5, CNDP2mitochondrial matrix, extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 6 routes

• tryptamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by AGXT2, SLC7A5, DDCmitochondrial matrix, extracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by ABAT, AGXT2, KYAT1mitochondrial matrix, cytosolOne of 5 routes

• (S)-2-hydroxyglutarateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, L2HGDHmitochondrial matrix

• 3-fumarylpyruvateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by ABAT, AGXT2, FAHD1mitochondrial matrix

• CHEBI:16856Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by AGXT2, AGXT, GSSmitochondrial matrix, cytosolOne of 3 routes

• glyoxylic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by AGXT2, AGXT, DAOmitochondrial matrix, peroxisome

• trans-1,2-dithiane-4,5-diolReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by AGXT2, SCLYmitochondrial matrix, cytosol