2-methyl-3-oxopropanoic acid

Three steps

• Met-tRNA(Met)Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by AGXT2, TYW1B, TYW1, MARS1mitochondrial matrix, cytosol

• (R)-glycerateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Serine:pyruvate aminotransferase
  3. D-glycerate:NADP+ 2-oxidoreductase

  Catalysed by AGXT2, AGXT, GRHPRmitochondrial matrix, cytosolOne of 4 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by AGXT2, TYW1B, TYW1, AADATmitochondrial matrix

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

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

• CHEBI:16857Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and threonine (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 7 routes

• ferrocytochrome cReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-Lactate:NAD+ oxidoreductase
  3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase

  Catalysed by AGXT2, LDHB, LDHAL6B, CYB5D1mitochondrial matrix, cytosolOne of 2 routes

• glycolaldehydeReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate carboxy-lyase (glycolaldehyde-forming)

  Catalysed by AGXT2, AGXTmitochondrial matrix, cytosolOne of 2 routes

• aldehydo-N-acetyl-D-mannosamineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by AGXT2, NPLmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 6 routes

• CHEBI:17203Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and proline (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 6 routes

• succinyl-4'-phosphopantetheineReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. succinyl-CoA + H2O = succinyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by ALDH6A1, MMUT, NUDT19, NUDT7 and 1 moremitochondrial matrix

• 17alpha-hydroxyprogesteroneReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxyprogesterone)

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 3 moremitochondrial matrix, extracellular space, cytosol, endoplasmic reticulum

• anilineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by ALDH6A1, ACOT7, NAT2, NAT1mitochondrial matrix, cytosol

• CHEBI:17312Reach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by ALDH6A1, ACOT7mitochondrial matrix, cytosolOne of 2 routes

• linoleic acidReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Active Transport
  3. Utilized Transport

  Catalysed by ALDH6A1mitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• sulfiteReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. transport of malate and sulfite (cytosol to mitochondria)

  Catalysed by AGXT2, ME2, SLC25A10mitochondrial matrix, cytosolOne of 2 routes

• enol-oxaloacetateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by AGXT2, ME2, SDHB, SDHAmitochondrial matrix

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Active Transport
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by ALDH6A1, CPT2, CPT1Amitochondrial matrix, cytosol, peroxisomeOne of 10 routes

• AH2Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by ABAT, AGXT2, MOCOSmitochondrial matrixOne of 7 routes

• 5-aminolevulinic acidReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by ALDH6A1, MMUT, ALAS1, ALAS2 and 1 moremitochondrial matrix

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

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 22 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by ALDH6A1, ACOT7, HADHA, ACADVL and 1 moremitochondrial matrix, cytosol

• Ala-tRNA(Ala)Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by ABAT, AGXT2, AARS1mitochondrial matrix, cytosol

• 4-methylumbelliferyl acetateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 5 moremitochondrial matrix, extracellular space, cytosol

• urateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-Lactate:NAD+ oxidoreductase
  3. transport of L-lactate and urate (cytosol to extracellular)

  Catalysed by AGXT2, LDHB, LDHAL6B, SLC22A12mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:17895Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and tyrosine (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 8 routes

• all-trans-retinalReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinoate reduction

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 3 moremitochondrial matrix, extracellular space, cytosol

• CHEBI:17927Reach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by ALDH6A1, ACOT7mitochondrial matrix, cytosol

• 3-phenylpyruvateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Glutamine:pyruvate aminotransferase
  3. 3-phenylpyruvate + L-glutamine = 2-oxoglutaramate + L-phenylalanine

  Catalysed by AGXT2, KYAT1mitochondrial matrix, cytosol

• fumaric acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by AGXT2, ME2, FHmitochondrial matrix, cytosolOne of 6 routes

• CHEBI:18019Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and lysine (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• thiocyanateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by AGXT2, MPST, TSTmitochondrial matrix, cytosol

• 4-fumarylacetoacetateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by AGXT2, FAHmitochondrial matrix, cytosol

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

  Catalysed by ABAT, AGXT2, SLC3A2, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 17 routes

• dTTPReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. pyruvate kinase (dTDP)
  3. DTTP Nucleotidohydrolase

  Catalysed by AGXT2, PKM, PKLR, ENTPD3 and 2 moremitochondrial matrix, cytosol, extracellular spaceOne of 11 routes

• CHEBI:18095Reach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Transport of Hydroxy-Proline

  Catalysed by ABAT, AGXT2, SLC1A4mitochondrial matrix, extracellular space, cytosol

• O-octanoyl-(R)-carnitineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Active Transport
  3. solute carrier (L-carnitine)

  Catalysed by ALDH6A1, SLC25A20mitochondrial matrix, cytosolOne of 7 routes

• S-methylmercury-L-cysteineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by AGXT2, TYW1B, TYW1, SLC7A8mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by AGXT2, BCKDHA, BCKDHBmitochondrial matrix

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

  Catalysed by ABAT, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-isoleucineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. D-serine = pyruvate + NH4(+)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

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

• N(omega),N('omega)-dimethyl-L-arginineReach 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

• (5Z)-dodecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by ALDH6A1, ACOT7, HADHA, ACAA2 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• (S)-lactoyl-AMPReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + H(+) = (S)-lactoyl-AMP + diphosphate

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 4 moremitochondrial matrix

• (S)-lactoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + CoA = (S)-lactoyl-CoA + AMP + diphosphate

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

• N-acetylneuraminate 9-phosphateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. pyruvate kinase (dTDP)
  3. N-acetylneuraminate synthase

  Catalysed by AGXT2, PKM, PKLR, NANSmitochondrial matrix, cytosol

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by ALDH6A1, ACOT7, HADHA, ACADVL and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by ALDH6A1, ACOT7, HADHA, ACADVL and 1 moremitochondrial matrix, cytosol