5-(3,3-dimethylguanidino)-2-oxopentanoate

Three steps

• homogentisateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. 4-Hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by AGXT2, AADAT, HPDcytosolOne of 2 routes

• CHEBI:16174Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. pyruvate kinase (dTDP)
  3. nucleoside-diphosphate kinase (dADP)

  Catalysed by AGXT2, PKM, PKLR, NME4 and 5 morecytosolOne of 3 routes

• sulfateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. transport of malate and sulfate (cytosol to mitochondria)

  Catalysed by AGXT2, ME2, SLC25A10mitochondrial matrix, cytosolOne of 8 routes

• ureaReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. L-Arginine amidinohydrolase

  Catalysed by AGXT2, SLC25A2, ARG1inner side of the membrane, outer side of the membrane, cytosolOne of 3 routes

• L-cystineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by AGXT2, SLC7A9, SLC3A1mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• CHEBI:16284Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. pyruvate kinase (dTDP)
  3. deoxythymidylate kinase (dADP)

  Catalysed by AGXT2, PKM, PKLR, DTYMKcytosol, mitochondrial matrixOne of 3 routes

• 11-cis-retinolReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by AGXT2, CS, DGAT1, LRAT and 3 moremitochondrial matrix, cytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)

  Catalysed by AGXT2, ABATmitochondrial matrixOne of 3 routes

• cis-aconitateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. cis-aconitate(in) + citrate(out) = cis-aconitate(out) + citrate(in)

  Catalysed by AGXT2, CS, SLC25A1mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-valineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and valine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 20 routes

• nitric oxideReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by AGXT2, SLC25A2, NOS3, NOS1 and 1 moreinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• CHEBI:16497Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. pyruvate kinase (dTDP)
  3. dGTP:pyruvate 2-O-phosphotransferase

  Catalysed by AGXT2, PKM, PKLRcytosol

• CHEBI:16523Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by AGXT2, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

• protein polypeptide chainReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. PROTEIN_BS

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 5 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosolOne of 2 routes

• CHEBI:16633Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AGXT2, SCLYmitochondrial matrix, cytosolOne of 2 routes

• Met-tRNA(Met)Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  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, MARS1cytosol

• (R)-glycerateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. L-Serine:pyruvate aminotransferase
  3. D-glycerate:NADP+ 2-oxidoreductase

  Catalysed by AGXT2, AGXT, GRHPRcytosolOne of 4 routes

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  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 more

• enol-phenylpyruvateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  3. 3-phenylpyruvate = enol-phenylpyruvate

  Catalysed by AGXT2, AADAT, MIF

• CHEBI:16856Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by AGXT2, GSScytosol

• CHEBI:16857Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and threonine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 17 routes

• glyoxylic acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by AGXT2, DAOperoxisomeOne of 5 routes

• ferrocytochrome cReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-Lactate:NAD+ oxidoreductase
  3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase

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

• N-acetyl-L-aspartateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by AGXT2, NAT8Lmitochondrial matrixOne of 2 routes

• oxalic acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by AGXT2, CS, SLC25A1mitochondrial matrix, cytosolOne of 3 routes

• glycolaldehydeReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate carboxy-lyase (glycolaldehyde-forming)

  Catalysed by AGXT2, AGXTcytosolOne of 2 routes

• aldehydo-N-acetyl-D-mannosamineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by AGXT2, NPLcytosolOne of 2 routes

• L-isoleucineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and isoleucine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 16 routes

• CHEBI:17203Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and proline (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 15 routes

• (S)-4-hydroxymandelateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by AGXT2, AADAT, HPDL

• L-glutamic 5-semialdehydeReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by AGXT2, SLC25A2, OATinner side of the membrane, outer side of the membrane, mitochondrial matrix

• 17alpha-hydroxyprogesteroneReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxyprogesterone)

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosol, endoplasmic reticulum

• anilineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by AGXT2, CS, NAT2, NAT1mitochondrial matrix, cytosol

• CHEBI:17312Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by AGXT2, CSmitochondrial matrix, cytosolOne of 2 routes

• sulfiteReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  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

• CHEBI:17380Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O
  3. Kynurenine-Oxoglutarate Transaminase

  Catalysed by AGXT2, KYAT3, AADATmitochondrial matrixOne of 2 routes

• agmatineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by AGXT2, SLC25A2inner side of the membrane, outer side of the membrane, mitochondrial matrix

• enol-oxaloacetateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by AGXT2, ME2, SDHB, SDHA

• AH2Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by AGXT2, MOCOSmitochondrial matrixOne of 7 routes

• 5-aminolevulinic acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by AGXT2, ALAS1, ALAS2, CPNE7mitochondrial matrix

• L-cysteineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and cysteine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 35 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by AGXT2, CS, HADHA, ACADVL and 1 moremitochondrial matrix

• Ala-tRNA(Ala)Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by AGXT2, AARS1mitochondrial matrix, cytosol

• 4-methylumbelliferyl acetateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  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 moreextracellular space, cytosol

• urateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  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. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and tyrosine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 18 routes

• all-trans-retinalReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinoate reduction

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosol

• CHEBI:17927Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by AGXT2, SLC25A2, SLC3A2, SLC7A6inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• fumaric acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by AGXT2, ME2, FHcytosolOne of 6 routes