2-methyl-3-oxopropanoic acid

Three steps

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by ALDH6A1, ACOT7, ECHS1, ACAA2 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by ALDH6A1, ACOT7, ECHS1, ACAA2 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• a quinolReach 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

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

  Catalysed by ALDH6A1mitochondrial matrix, cytosol, endoplasmic reticulum

• CHEBI:27540Reach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by ALDH6A1, ACOT7, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• selenomethionineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by AGXT2, CTHmitochondrial matrix, cytosol

• CHEBI:27760Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by AGXT2, CTHmitochondrial matrix, cytosol

• elaidic acidReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (elaidate)

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

• 3-mercaptolactic acidReach 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-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by AGXT2, MPST, LDHB, LDHC and 2 moremitochondrial matrix, cytosol

• 2-oxopentanoateReach 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. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by AGXT2mitochondrial matrix

• CHEBI:28701Reach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by ALDH6A1, ACOT7, ACAA1, SCP2 and 1 moremitochondrial matrix, cytosol, peroxisomeOne of 7 routes

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

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

• pyridoxine 5'-phosphateReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by ABAT, AGXT2, SCLYmitochondrial matrix, cytosol

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

  Catalysed by AGXT2, NPLmitochondrial matrix, cytosol

• a carboxylateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1mitochondrial matrix

• chlorine(.)Reach 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 chloride (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 7 routes

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

  Catalysed by AGXT2, MPSTmitochondrial matrix

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

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 8 routes

• citric acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by AGXT2, PC, CSmitochondrial matrixOne of 9 routes

• (9Z)-octadecenoateReach 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

• isocitric acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. pyruvate kinase (dTDP)
  3. transport of isocitrate and PEP (cytosol to mitochondria)

  Catalysed by AGXT2, PKM, PKLR, SLC25A1mitochondrial matrix, cytosolOne of 5 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, PC, GOT2mitochondrial matrix

• (9Z)-hexadecenoateReach 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 6 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach 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

• cis-aconitic acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. pyruvate kinase (dTDP)
  3. transport of cis-aconitate and PEP (cytosol to mitochondria)

  Catalysed by AGXT2, PKM, PKLR, SLC25A1mitochondrial matrix, cytosolOne of 5 routes

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

  Catalysed by AGXT2, ME2, SLC25A10mitochondrial matrix, cytosolOne of 4 routes

• 4-methylthio-2-oxobutanoic acidReach 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:oxygen oxidoreductase (deaminating)

  Catalysed by AGXT2, TYW1B, TYW1, IL4I1mitochondrial matrix, cytosol

• 2-oxohexanoateReach 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. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by AGXT2mitochondrial matrix

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

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

• L-alpha-aminobutyric acidReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by AGXT2, GPTmitochondrial matrix, cytosol

• 5-aminolevulinateReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by ALDH6A1, MMUT, ALAS1, ALAS2mitochondrial matrix

• glyoxylateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)

  Catalysed by AGXT2, ME2, CLYBLmitochondrial matrixOne of 8 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate

  Catalysed by ALDH6A1, MMUT, SUGCTmitochondrial matrixOne of 2 routes

• 13-cis-retinalReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

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

• L-ornithineReach 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 ornithine (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 7 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

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

• gamma-Glu-AlaReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. gamma-glutamyltransferase (alanine)

  Catalysed by ABAT, AGXT2, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosol

• taurineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by ALDH6A1, ACOT7, BAATmitochondrial matrix, cytosolOne of 2 routes

• acetoacetyl-CoAReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. acetoacetate + succinyl-CoA = acetoacetyl-CoA + succinate

  Catalysed by ALDH6A1, MMUT, OXCT1mitochondrial matrixOne of 3 routes

• glycineReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Alanine:glyoxylate aminotransferase

  Catalysed by ABAT, AGXT2, AGXTmitochondrial matrixOne of 24 routes

• (S)-malyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by AGXT2, ME2, SUCLG1, SUCLA2mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ALDH6A1, ACOT7, ACP2mitochondrial matrix, cytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by ALDH6A1, ACOT7mitochondrial matrix, cytosolOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ALDH6A1, ACOT7, ACAA1, MYO5Bmitochondrial matrix, cytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of butyrate and pyruvate (cytosol to extracellular)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 9 moremitochondrial matrix, extracellular space, cytosolOne of 13 routes

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. glutarate + succinyl-CoA = glutaryl-CoA + succinate

  Catalysed by ALDH6A1, MMUT, SUGCTmitochondrial matrixOne of 3 routes

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. itaconate + succinyl-CoA = itaconyl-CoA + succinate

  Catalysed by ALDH6A1, MMUT, SUGCTmitochondrial matrixOne of 3 routes