(R)-3-aminoisobutyric acid

Three steps

• di-mu-sulfido-diiron(2+)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by AGXT2, SLC7A5, NDUFAB1, FDXR and 5 moremitochondrial matrix, extracellular space, cytosol

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by AGXT2, SLC7A5, BCAT2, BCAT1mitochondrial matrix, extracellular space, cytosol

• 5-aminolevulinateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by AGXT2, AGXT, ALAS1, ALAS2mitochondrial matrix

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

  Catalysed by AGXT2, SLC7A5, BCAT1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, SLC7A5, GOT2, GOT1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• glycochenodeoxycholateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by AGXT2, AGXT, BAATmitochondrial matrix

• glycochenodeoxycholic acidReach 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

• 3-hydroxyanthranilateReach 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 + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by AGXT2, KYNUmitochondrial matrix

• glyoxylateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by AGXT2, AGXT, AADATmitochondrial matrixOne of 11 routes

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

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by AGXT2, AGXT, GCATmitochondrial matrix

• (R)-lactic acidReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by ABAT, AGXT2, LDHDmitochondrial matrix, cytosol

• (S)-lactic acidReach 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+ oxidoreductase

  Catalysed by ABAT, AGXT2, LDHB, LDHAL6Bmitochondrial matrixOne of 5 routes

• 25-hydroxycholesterolReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by AGXT2, MOCOS, CH25Hmitochondrial matrix

• 2-iminopropanoateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by ABAT, AGXT2, RIDAmitochondrial matrix

• phosphoenolpyruvic acidReach 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

• N-acetyl-beta-neuraminic acidReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by ABAT, AGXT2, NPLmitochondrial matrix, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by AGXT2, AGXT, GLDC, GCSHmitochondrial matrix

• taurineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by AGXT2, BAATmitochondrial matrix, cytosolOne of 4 routes

• all-trans-13,14-dihydroretinolReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by AGXT2, MOCOS, RETSATmitochondrial matrix

• 3,3',5-triiodo-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',5-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO2mitochondrial matrix

• 3,3',5'-triiodo-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',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3mitochondrial matrix

• a 3-acylpyruvateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by ABAT, AGXT2, FAHD1mitochondrial matrix

• succinyl-CoAReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  3. (R)-methylmalonyl-CoA = succinyl-CoA

  Catalysed by ABAT, AGXT2, ALDH6A1, MMUTmitochondrial matrix

• (S)-methylmalonyl-CoAReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  3. propanoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by ABAT, AGXT2, ALDH6A1, PCCA and 1 moremitochondrial matrixOne of 2 routes

• 3'-dephospho-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by AGXT2, ACP2mitochondrial matrix, lysosomeOne of 2 routes

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

  Catalysed by AGXT2mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

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

• (6Z,9Z,12Z)-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 (gamma-linolenate)

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

• acryloyl-CoAReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by ABAT, AGXT2, ALDH6A1, ACADS and 1 moremitochondrial matrix

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

  Catalysed by AGXT2, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-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 (lauric acid)

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

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

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

• eicosanoyl-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 (eicosanoate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 12 routes

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

  Catalysed by AGXT2, SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-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)-octadecadienoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

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

• octanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by AGXT2, CROTmitochondrial matrixOne of 20 routes

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

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-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 (stearate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 4 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin H2Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by AGXT2, MOCOS, PTGS1, PTGS2mitochondrial matrixOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by AGXT2, MOCOS, TBXAS1, PTGS2 and 1 moremitochondrial matrix

• sarcosineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by AGXT2, AGXT, PIPOXmitochondrial matrixOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by AGXT2, AGXT, SHMT1, SHMT2mitochondrial matrix

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by AGXT2, SLC7A6, ASLmitochondrial matrix, extracellular space, cytosolOne of 6 routes

• carnosineReach 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 + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by AGXT2, SLC7A10, CARNS1mitochondrial matrix, inner side of the membrane, outer side of the membrane

• 4-guanidinobutanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

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

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

  Catalysed by AGXT2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by AGXT2, ADSS2, ADSS1mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by AGXT2, SLC7A6, GATMmitochondrial matrix, extracellular space, cytosolOne of 2 routes