2-oxobutanoate

Three steps

• fumaric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by AGXT2, CLYBL, FHcytosolOne of 6 routes

• CHEBI:18019Reach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. transport of glycine and lysine (cytosol to extracellular)

  Catalysed by SDS, SDSL, SHMT1, SLC7A6extracellular space, cytosolOne of 8 routes

• thiocyanateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by AGXT2, MPST, TSTcytosol

• 4-fumarylacetoacetateReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 5 morecytosol, extracellular space

• dTTPReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. pyruvate kinase (dTDP)
  3. DTTP Nucleotidohydrolase

  Catalysed by AGXT2, PKM, PKLR, ENTPD3 and 2 morecytosol, extracellular spaceOne of 11 routes

• CHEBI:18095Reach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. Transport of Hydroxy-Proline

  Catalysed by SDS, SDSL, SLC7A10, SLC1A4inner side of the membrane, outer side of the membrane, extracellular space, cytosol

• O-octanoyl-(R)-carnitineReach from here →
  1. 2-oxobutyrate oxidation
  2. Active Transport
  3. solute carrier (L-carnitine)

  Catalysed by SLC25A20cytosol, mitochondrial matrixOne of 7 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by SDS, SDSL, SHMT1, GARTcytosol

• CHEBI:18405Reach from here →
  1. Selenocystathionine Lysteine-lyase (deaminating)
  2. Selenocystathionine Lysteine-lyase (deaminating)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by CTH, SCLYcytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by SDS, SDSL, SLC1A5, PFASinner side of the membrane, outer side of the membrane, cytosol

• ophthalmateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by SDS, SDSL, SHMT1, GSSOne of 2 routes

• S-methylmercury-L-cysteineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  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, SLC7A8inner side of the membrane, outer side of the membraneOne of 2 routes

• 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)Reach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(out) + glutarate(in) = 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(in) + glutarate(out)

  Catalysed by AADAT, SLC22A6, SLC22A24inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-aminoisobutanoateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by SDS, SDSL, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• D-isoleucineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. D-serine = pyruvate + NH4(+)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by AGXT2, SRR, SLC7A10inner side of the membrane, outer side of the membrane

• 3-carboxy-4-methyl-5-propyl-2-furanpropanoateReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(out) + a dicarboxylate(in) = 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(in) + a dicarboxylate(out)

  Catalysed by AADAT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• L-sepiapterinReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. L-sepiapterin(out) + a dicarboxylate(in) = L-sepiapterin(in) + a dicarboxylate(out)

  Catalysed by AADAT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by SDS, SDSL, SHMT1, AGXT2One of 4 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. Selenocystathionine Lysteine-lyase (deaminating)
  2. L-Serine hydro-lyase (adding homocysteine)
  3. Methionine Synthase

  Catalysed by CTH, CBScytosol, nucleusOne of 4 routes

• (5Z)-dodecenoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by ACOT7, HADHA, ACAA2, HADHBcytosol, mitochondrial matrixOne of 2 routes

• (S)-lactoyl-AMPReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + H(+) = (S)-lactoyl-AMP + diphosphate

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 4 more

• (S)-lactoyl-CoAReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  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 more

• N-acetylneuraminate 9-phosphateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. pyruvate kinase (dTDP)
  3. N-acetylneuraminate synthase

  Catalysed by AGXT2, PKM, PKLR, NANScytosol

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by ACOT7, HADHA, ACADVL, HADHBcytosol, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by ACOT7, HADHA, ACADVL, HADHBcytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by ACOT7, ECHS1, ACAA2, HADHcytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by ACOT7, ECHS1, ACAA2, HADHcytosol, mitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by SDS, SDSL, SHMT1, GLYAT and 2 moremitochondrial matrix

• a quinolReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. trans-4-hydroxy-L-proline(out) + L-threonine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-threonine(out) + Na(+)(in)
  3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)

  Catalysed by SDS, SDSL, SLC1A4, PRODH2inner side of the membrane, outer side of the membraneOne of 2 routes

• alpha-linolenic acidReach from here →
  1. 2-oxobutyrate oxidation
  2. Active Transport
  3. Utilized Transport

  No catalyst recordedcytosol, mitochondrial matrix, endoplasmic reticulum

• phenylacetylglycineReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by SDS, SDSL, SHMT1, GLYATcytosolOne of 3 routes

• CHEBI:27540Reach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by ACOT7, ACSM4, ACSM1, ACSM2A and 3 morecytosolOne of 6 routes

• elaidic acidReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (elaidate)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (2-hydroxyphenyl)acetic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  3. Phenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by AGXT2, AADAT, HPDcytosol

• 3-mercaptolactic acidReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  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 morecytosol

• CHEBI:28666Reach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. LTD4 hydrolysis

  Catalysed by SDS, SDSL, SHMT1cytosol

• CHEBI:28701Reach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by ACOT7, ACAA1, SCP2, MYO5Bcytosol, peroxisomeOne of 7 routes

• 17alpha-hydroxypregnenoloneReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxypregnenolone)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 5 morecytosol, extracellular space, endoplasmic reticulum

• pyridoxine 5'-phosphateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SDS, SDSL, SLC7A10, SCLYinner side of the membrane, outer side of the membrane, cytosol

• N-glycoloylneuraminateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CMP-N-acetylneuraminate monooxygenase (N-acetylneuraminate)

  Catalysed by AGXT2, NPLcytosol

• a carboxylateReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1

• L-ascorbic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. N-octanoyl-(2S)-hydroxyglycine = octanamide + glyoxylate
  3. N-octanoylglycine + 2 L-ascorbate + O2 = N-octanoyl-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by AGXT2, PAMOne of 6 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)
  2. lipoamide to thiamin-PP conversion
  3. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

  Catalysed by BCKDHA, BCKDHB, DBTmitochondrial matrixOne of 2 routes

• Gly-tRNA(Gly)Reach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by SDS, SDSL, SHMT1, GARS1cytosol

• Gln-tRNA(Gln)Reach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)
  3. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

  Catalysed by SDS, SDSL, SLC1A5, QARS1inner side of the membrane, outer side of the membrane, cytosol

• chlorine(.)Reach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 5 morecytosol, extracellular spaceOne of 11 routes

• glycocholateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by SDS, SDSL, SHMT1, BAATperoxisomeOne of 5 routes

• hydrogen sulfideReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  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, MPST