2-methyl-3-oxopropanoic acid

Three steps

• dTMPReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. pyruvate kinase (dTDP)
  3. DTDP Diphosphohydrolase

  Catalysed by AGXT2, PKM, PKLR, ENTPD3 and 2 moremitochondrial matrix, cytosol, extracellular spaceOne of 5 routes

• CHEBI:63544Reach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

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

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

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 10 routes

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

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

• Mo-molybdopterinReach 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 matrix

• (2E)-octadecenoyl-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 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

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

• (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Active Transport
  3. Utilized Transport

  Catalysed by ALDH6A1mitochondrial matrix, cytosol, endoplasmic reticulum

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (EPA)

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

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (DPA)

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

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (linolenate)

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

• (13Z)-docosenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

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

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

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

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

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-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,12Z,15Z,18Z,21Z)-THA)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

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

• (17Z)-hexacosenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

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

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

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

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (DHA)

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

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

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

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

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

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

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

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

  Catalysed by ABAT, AGXT2mitochondrial matrixOne of 6 routes

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

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

• (11Z)-octadecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by ALDH6A1, ACOT7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Activation of dodecanedioic acid

  Catalysed by ALDH6A1, ACOT7mitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Production of Sebacoylcarnitine

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

• octanedioyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Production of Suberylcarnitine

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

• hexanedioyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by ALDH6A1, ACOT7mitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

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

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

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

• 2-iminobutanoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 2-iminobutanoate + H2O = 2-oxobutanoate + NH4(+)

  Catalysed by AGXT2, RIDAmitochondrial matrix

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by ALDH6A1, ACOT7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

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

• 2-aminoprop-2-enoateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. D-serine = pyruvate + NH4(+)
  3. D-serine = 2-aminoprop-2-enoate + H2O

  Catalysed by AGXT2, SRRmitochondrial matrix

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by ALDH6A1, ACOT7, CRAT, CROTmitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by ALDH6A1, ACOT7, ACAA1, HSD17B4 and 2 moremitochondrial matrix, cytosol, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. Production of Hexadecanedioylcarnitine

  Catalysed by ALDH6A1, ACOT7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by ALDH6A1, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 7 routes

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by ALDH6A1, ACOT7, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 3 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

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

• O-4,8-dimethylnonanoyl-(R)-carnitineReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Active Transport
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by ALDH6A1, CRAT, CROTmitochondrial matrix, cytosol, peroxisomeOne of 5 routes

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

  Catalysed by ALDH6A1, ACOT7, ACADM, ECHS1 and 2 moremitochondrial matrix, cytosol

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. 2-Methyl-3-oxopropanoate:NAD+ oxidoreductase (CoA-propanoylating)
  2. Propanoyl-CoA hydrolase
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by ALDH6A1, ACOT7, HADHA, ACAA2 and 1 moremitochondrial matrix, cytosol

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

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

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

  Catalysed by ALDH6A1, ACOT7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• thromboxane B2Reach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. thromboxane B2(out) + 2 (S)-lactate(in) = thromboxane B2(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