(R)-3-aminoisobutyric acid

Three steps

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

  Catalysed by AGXT2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

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

• an N-acylglycineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by AGXT2, AGXT, GLYATL3, GLYAT and 1 moremitochondrial matrix

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by ABAT, AGXT2, MPSTmitochondrial matrixOne of 5 routes

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

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 2 routes

• guanidinoacetateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. L-Arginine:glycine amidinotransferase

  Catalysed by AGXT2, AGXT, GATMmitochondrial matrix, cytosolOne of 5 routes

• sphing-4-enineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by AGXT2, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

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

  Catalysed by AGXT2, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

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

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

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

  Catalysed by AGXT2, AGXT, GSSmitochondrial matrixOne of 2 routes

• L-saccharopineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

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

• leukotriene C4Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. leukotriene C4 hydrolysis

  Catalysed by AGXT2, AGXTmitochondrial matrix, cytosol

• dimethylamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by AGXT2, DDAH1mitochondrial matrix, cytosolOne of 2 routes

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

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

• L-glutamate 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-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

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

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by AGXT2, AGAmitochondrial matrix

• L,L-cystathionineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by AGXT2, NAGSmitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

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

• 6-amino-2-oxohexanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by AGXT2, SLC7A6mitochondrial matrix, extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by AGXT2, SLC7A5, BHMT2mitochondrial matrix, extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by AGXT2, SLC7A5, BHMT2mitochondrial matrix, extracellular space, cytosol

• glutathione disulfideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  3. cystine to cysteine conversion

  Catalysed by AGXT2, SLC7A9, SLC3A1mitochondrial matrix, extracellular space, cytosol

• L-tyrosineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by AGXT2, AGXT, AADATmitochondrial matrixOne of 4 routes

• an acyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by AGXT2, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

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

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

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

  Catalysed by AGXT2, KYNU, AADATmitochondrial matrixOne of 2 routes

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by AGXT2, PAICSmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by AGXT2, KYNU, KYAT3, AADATmitochondrial matrixOne of 13 routes

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

  Catalysed by AGXT2, KYNU, AADATmitochondrial matrixOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by AGXT2, SLC7A6, IL4I1mitochondrial matrix, extracellular space, cytosol

• (3Z)-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-5-enoyl-CoA)

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

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

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

• N-formyl-L-kynurenineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by AGXT2, SLC7A5, IDO1, TDO2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (3S)-citramalyl-CoAReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by ABAT, AGXT2, CLYBLmitochondrial matrix

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

  Catalysed by AGXT2, KYNU, AADATmitochondrial matrix

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by AGXT2, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

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

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

• L-methionine (S)-S-oxideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

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

• L-methionine (R)-S-oxideReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by AGXT2, SLC7A5, MSRB3, MSRB1 and 1 moremitochondrial matrix, extracellular space, cytosol

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

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by AGXT2, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Alanine:glyoxylate aminotransferase
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrixOne of 2 routes

• methylamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

  Catalysed by AGXT2, DDAH1mitochondrial matrix

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

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

• pyridoxal 5'-phosphateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by AGXT2, SCLY, PNPOmitochondrial matrix, cytosol

• an L-alpha-amino acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

  Catalysed by AGXT2, KYNU, SLC7A11mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of alanine and chloride (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by AGXT2, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach 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 2 routes