2-methyl-3-oxopropanoic acid

Three steps

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 13 routes

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ALDH6A1, ACOT7, CROTmitochondrial matrix, cytosolOne of 20 routes

• phytanoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. phytanate-CoA ligase

  Catalysed by ALDH6A1, ACOT7mitochondrial matrix, cytosolOne of 5 routes

• propanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
  3. 2-methyl-3-oxopropanoate + NAD(+) + CoA + H2O = propanoyl-CoA + hydrogencarbonate + NADH + H(+)

  Catalysed by AGXT2, ALDH6A1mitochondrial matrixOne of 2 routes

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 5 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin E1Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin E1(out) + 2 (S)-lactate(in) = prostaglandin E1(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin F2alpha(out) + 2 (S)-lactate(in) = prostaglandin F2alpha(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin H2Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin H2(out) + 2 (S)-lactate(in) = prostaglandin H2(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin D2Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin D2(out) + 2 (S)-lactate(in) = prostaglandin D2(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate

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

• O-acetyl-(R)-carnitineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Active Transport
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by ALDH6A1, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 9 routes

• sphing-4-enineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ALDH6A1, ACOT7, ASAH1, ACER2 and 2 moremitochondrial matrix, cytosol, Golgi apparatusOne of 2 routes

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

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

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

  Catalysed by AGXT2, NPL, CMASmitochondrial matrix, cytosol, nucleusOne of 2 routes

• spermidineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by ALDH6A1, ACOT7, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

• L-tryptophanReach 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 tryptophan (cytosol to extracellular)

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

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

  Catalysed by ABAT, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 5 routes

• L-kynurenineReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by ABAT, AGXT2, KYNUmitochondrial matrix

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

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

• 2-hydroxy-3-oxopropanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate aldose-ketose-isomerase

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

• L-asparagineReach 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 asparagine (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 10 routes

• L-phenylalanineReach 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 phenylalanine (cytosol to extracellular)

  Catalysed by ABAT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 12 routes

• 3-hydroxy-L-kynurenineReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by ABAT, AGXT2, KYNUmitochondrial matrix

• L,L-cystathionineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by ALDH6A1, ACOT7, NAGSmitochondrial matrix, cytosol

• L-homocysteineReach 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-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by AGXT2, TYW1B, TYW1, BHMT2mitochondrial matrix

• S-methyl-L-methionineReach 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-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by AGXT2, TYW1B, TYW1, BHMT2mitochondrial matrix

• N-acetylputrescineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetylputrescine + H2O = putrescine + acetate

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

• (2R)-2-phosphoglycerateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. pyruvate kinase (dTDP)
  3. 2-phospho-D-glycerate hydro-lyase (phosphoenolpyruvate-forming)

  Catalysed by AGXT2, PKM, PKLR, ENO1 and 2 moremitochondrial matrix, cytosol

• 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 7 routes

• an acyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ALDH6A1, ACOT7, ASAH1, ACER2 and 2 moremitochondrial matrix, cytosol, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by ABAT, AGXT2, SLC1A4mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• kynurenateReach 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 + L-kynurenine = kynurenate + L-cysteine + H2O

  Catalysed by AGXT2, MPST, AADATmitochondrial matrixOne of 2 routes

• N(8)-acetylspermidineReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N(8)-acetylspermidine + H2O = spermidine + acetate

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

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

  Catalysed by ALDH6A1, ACOT7, HADHA, ACAA2 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, SLC27A5mitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes

• phosphoenolpyruvateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by AGXT2, PC, PCK1, PCK2mitochondrial matrix

• an N-acetyl-L-cysteine-S-conjugateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. an N-acetyl-L-cysteine-S-conjugate + H2O = an S-substituted L-cysteine + acetate

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

• L-methionine (S)-S-oxideReach 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. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by AGXT2, TYW1B, TYW1, MSRAmitochondrial matrix

• L-methionine (R)-S-oxideReach 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. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by AGXT2, TYW1B, TYW1, MSRB3 and 2 moremitochondrial matrix

• deoxycholoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• (2S)-2-phospholactateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (2S)-2-phospholactate + H2O = (S)-lactate + phosphate

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moremitochondrial matrixOne of 2 routes

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

  Catalysed by ABAT, AGXT2, SLC1A4mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin E2(out) + 2 (S)-lactate(in) = prostaglandin E2(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• decanoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ALDH6A1, ACOT7, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Carnitine O-Palmitoyltransferase

  Catalysed by ALDH6A1, ACOT7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• L-cysteinylglycineReach 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, GGTLC3, GGT1 and 4 moremitochondrial matrix, cytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by ALDH6A1, ACOT7, CPT1A, CPT1Bmitochondrial matrix, cytosol