(R)-3-aminoisobutyric acid

Three steps

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

  Catalysed by AGXT2, MOCOS, DIO3mitochondrial matrix

• 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, DIO2mitochondrial matrixOne of 2 routes

• 3,3'-diiodothyronamineReach 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'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

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

• 3',5'-diiodothyronamineReach 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'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO3mitochondrial matrix

• 3,3',5'-triiodothyronamineReach 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'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO3mitochondrial matrix

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-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 (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-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 Trans (C18:2->C16:2), Mitochondrial

  Catalysed by AGXT2, HADHA, ACADVL, HADHBmitochondrial matrix

• (4Z,7Z)-decadienoyl-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 (C12:3->C10:2), Mitochondrial

  Catalysed by AGXT2, ECHS1, ACAA2, HADHmitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-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 (C10:2->C8:1), Mitochondrial

  Catalysed by AGXT2, ECHS1, ACAA2, HADHmitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by AGXT2, AGXT, GLYAT, GLYATL2 and 1 moremitochondrial matrixOne of 3 routes

• a quinolReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)
  3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)

  Catalysed by AGXT2, SLC1A4, PRODH2mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by AGXT2, SLC6A14, LPOmitochondrial matrix, extracellular space, cytosolOne of 4 routes

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

  Catalysed by AGXT2, SLC6A14, SLC12A2, SLC12A1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• phenylacetylglycineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

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

• CHEBI:27540Reach 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 (hexanoic acid)

  Catalysed by AGXT2, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 6 routes

• CHEBI:27760Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by AGXT2, SCLY, CTHmitochondrial matrix, cytosol

• elaidic acidReach 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 (elaidate)

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

• 5-hydroxytryptophanReach 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 2 routes

• CHEBI:28666Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. LTD4 hydrolysis

  Catalysed by AGXT2, AGXTmitochondrial matrix, cytosol

• CHEBI:28701Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by AGXT2, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 7 routes

• 3-disulfanyl-L-alanineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by AGXT2, SLC7A9, SLC3A1, CTHmitochondrial matrix, extracellular space, cytosol

• N-acetyl-L-cysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

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

• K(+)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by AGXT2, SLC7A5, SLC1A1mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

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

  Catalysed by AGXT2, SLC7A5, CARS1mitochondrial matrix, extracellular space, cytosol

• Phe-tRNA(Phe)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by AGXT2, SLC7A5, FARSB, FARSAmitochondrial matrix, extracellular space, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by AGXT2, AGXT, GARS1mitochondrial matrix, cytosol

• Asp-tRNA(Asp)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by AGXT2, DARS1mitochondrial matrix, cytosol

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

  Catalysed by AGXT2, SLC7A5, WARS1mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by AGXT2, SLC7A5, IARS1mitochondrial matrix, extracellular space, cytosol

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

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

• isocyanateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by AGXT2, SLC7A6, LACC1mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by AGXT2, SLC7A5, NARS1mitochondrial matrix, extracellular space, cytosol

• hydrogenselenideReach 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-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)

  Catalysed by AGXT2, MOCOS, SCLYmitochondrial matrix

• glycocholateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by AGXT2, AGXT, BAATmitochondrial matrix, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

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

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

  Catalysed by ABAT, AGXT2, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:30249Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by AGXT2, SLC7A5, IDO1, TDO2 and 1 moremitochondrial matrix, extracellular space, cytosol

• formic 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 formate and pyruvate (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

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

  Catalysed by AGXT2, KYNUmitochondrial matrix, cytosol

• propionic 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 propanoate and pyruvate (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• butyric 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 butyrate and pyruvate (cytosol to extracellular)

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

• malonic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and chloride (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

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

• 3-hydroxypyruvic acidReach 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 aminotransferase

  Catalysed by ABAT, AGXT2, AGXTmitochondrial matrix, cytosolOne of 2 routes

• L-lysineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and leucine (cytosol to extracellular)
  3. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)

  Catalysed by AGXT2, SLC7A5, SLC7A7mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• D-lysineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by AGXT2, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• putrescineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by AGXT2, SLC7A6, SLC3A2, SLC7A7mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• D-arginineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by AGXT2, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by AGXT2, CADmitochondrial matrix

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

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 2 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by AGXT2, SLC7A5, IL4I1mitochondrial matrix, extracellular space, cytosol