(R)-3-aminoisobutyric acid

Three steps

• N-acetyl-L-aspartateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by AGXT2, NAT8Lmitochondrial matrixOne of 2 routes

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

  Catalysed by AGXT2, SLC6A14, SLC26A6mitochondrial matrix, extracellular space, cytosol

• (R)-3-hydroxybutyric acidReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of (R)-3-hydroxybutanoate and pyruvate (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

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

  Catalysed by ABAT, AGXT2, AGXTmitochondrial matrixOne of 2 routes

• L-glutamic 5-semialdehydeReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

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

• anilineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by AGXT2, NAT2, NAT1mitochondrial matrix, cytosol

• aceneuramateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by ABAT, AGXT2, NPLmitochondrial matrix

• CHEBI:17312Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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 ABAT, AGXT2, TYW1B, TYW1mitochondrial matrixOne of 5 routes

• CHEBI:17380Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by AGXT2, SLC7A5, PRODH2mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• agmatineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by AGXT2, SLC7A6mitochondrial matrix, extracellular space, cytosol

• 5-aminolevulinic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by AGXT2, AGXT, ALAS1, ALAS2 and 1 moremitochondrial matrix

• indole-3-pyruvateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by AGXT2, SLC7A5, IL4I1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, DIO1mitochondrial matrix

• 3,3'-diiodo-L-thyronineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, DIO3mitochondrial matrixOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  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, DIO1mitochondrial matrixOne of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-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 (C18:3->C16:3), Mitochondrial

  Catalysed by AGXT2, HADHA, ACADVL, HADHBmitochondrial matrix

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

  Catalysed by AGXT2, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

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

• 5-hydroxy-L-tryptophanReach 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, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, SLC7A5, BCAT2mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• CHEBI:17927Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 2 routes

• 3-oxopropanoic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

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

• 3-phenylpyruvateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by AGXT2, SLC7A5, GOT2, TATmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• dTDPReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate kinase (dTDP)

  Catalysed by ABAT, AGXT2, PKM, PKLRmitochondrial matrix, cytosol

• CHEBI:18183Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by AGXT2, GGT1, GGT5, GGTLC2 and 5 moremitochondrial matrix, extracellular space, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and chloride (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by AGXT2, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by AGXT2, AGXT, GARTmitochondrial matrix, cytosol

• 2-phenylethylamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

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

• CHEBI:18405Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by AGXT2, SCLY, PNPOmitochondrial matrix, cytosolOne of 3 routes

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by AGXT2, GCLCmitochondrial matrix

• ophthalmateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by AGXT2, AGXT, GSSmitochondrial matrix

• S-methylmercury-L-cysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by AGXT2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• D-isoleucineReach 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-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membrane

• Xaa-L-Pro dipeptideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)
  3. Xaa-L-Pro dipeptide + H2O = an L-alpha-amino acid + L-proline

  Catalysed by AGXT2, SLC1A4, PEPDmitochondrial matrix, inner side of the membrane, outer side of the membrane

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

  Catalysed by AGXT2, MOCOS, DIO3mitochondrial matrix

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 7 routes

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 3 routes

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 3 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by AGXT2, AGXT, SHMT1mitochondrial matrix, cytosolOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by AGXT2, SLC7A5, CARS2mitochondrial matrix, extracellular space, cytosol

• (5Z)-dodecenoyl-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-oxomyrist-7-enoyl-CoA)

  Catalysed by AGXT2, HADHA, ACAA2, HADHBmitochondrial matrixOne of 2 routes

• 3-iodothyronamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3mitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach 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-cetoleic acid)

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

• 3-iodo-L-thyronineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3mitochondrial matrixOne of 2 routes

• (7Z)-tetradecenoyl-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 ((7Z)-tetradecenoic acid)

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

• 3'-iodo-L-thyronineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO2mitochondrial matrixOne of 2 routes

• L-thyronineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO3mitochondrial matrix