guanidinoacetate

Three steps

• quinolinic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of Quinolinic Acid

  Catalysed by SLC26A6, SLC22A6extracellular space, cytosolOne of 2 routes

• cyanamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. urea = cyanamide + H2O

  Catalysed by GATM, ARG1, CA1, CA2cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by GATM, SLC7A6, AADATcytosol, extracellular spaceOne of 2 routes

• creatinineReach from here →
  1. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase
  2. creatine kinase (creatine)
  3. creatine-phosphate to creatinine conversion

  Catalysed by GAMT, CKM, CKB, CKMT1A and 1 morecytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by GATM, SLC7A6, CNDP2cytosol, extracellular space

• 2-oxobutanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and threonine (cytosol to extracellular)
  3. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by GATM, SLC7A6, SDS, SDSLcytosol, extracellular spaceOne of 5 routes

• tryptamineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by GATM, SLC7A6, DDCcytosol, extracellular space

• 2-oxoglutaramateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by GATM, SLC7A6, KYAT1cytosol, extracellular spaceOne of 3 routes

• formaldehydeReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. sarcosine:oxygen oxidoreductase (demethylating)

  Catalysed by GATM, GNMT, PIPOX, SARDHcytosol, peroxisomeOne of 2 routes

• bilirubin IXalphaReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of bilirubin and GSH (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of (R)-3-hydroxybutanoate and formate (cytosol to extracellular)

  Catalysed by SLC26A4, SLC16A4, SLC16A5, SLC16A7 and 1 moreextracellular space, cytosolOne of 2 routes

• taurolithocholateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and taurolithocholate (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 4 routes

• CHEBI:17208Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by GATM, GSScytosol

• homocysteineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Formation of Homocystine

  Catalysed by GATM, GSScytosol

• L-glutamic 5-semialdehydeReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by GATM, ARG1, OATcytosol, mitochondrial matrix

• anilineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by GATM, BAAT, NAT2, NAT1cytosol, peroxisome

• CHEBI:17312Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by GATM, BAATcytosol, peroxisomeOne of 2 routes

• linoleic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A14extracellular space, cytosol, endoplasmic reticulumOne of 2 routes

• sulfiteReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by GATM, GSS, TSTD1cytosolOne of 6 routes

• D-aspartic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by SLC12A2, SLC12A1, SLC1A7, SLC1A3 and 3 moreextracellular space, cytosol

• CHEBI:17380Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by GATM, SLC7A6, SLC7A5cytosol, extracellular spaceOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by GATM, SLC7A6, PRODH2cytosol, extracellular spaceOne of 2 routes

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Carnitine Transport by Atb0
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by SLC6A14, CPT2, CPT1Aextracellular space, cytosol, peroxisomeOne of 10 routes

• AH2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by GATM, SLC7A6, MOCOScytosol, extracellular spaceOne of 6 routes

• S-methyl-5'-thioadenosineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by GATM, SLC3A2, SLC7A7, SRMcytosol, extracellular spaceOne of 2 routes

• diiodineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and iodide (cytosol to extracellular)
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by SLC26A4, TPOextracellular space, cytosolOne of 2 routes

• nitrateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

  Catalysed by GATM, NOS3, NOS1, NOS2 and 1 morecytosol

• indole-3-pyruvateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by GATM, SLC7A6, IL4I1cytosol, extracellular spaceOne of 3 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Steryl-Sulfatase

  Catalysed by SLC26A6, STSextracellular space, cytosol, Golgi apparatusOne of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by GATM, BAAT, HADHA, ACADVL and 1 morecytosol, peroxisome, mitochondrial matrix

• CHEBI:17724Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by GATM, GNMT, PDPR, DMGDHcytosolOne of 2 routes

• Ala-tRNA(Ala)Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by GATM, SLC7A6, AARS1cytosol, extracellular space

• CHEBI:17769Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by GATM, SLC3A2, SLC7A7, AOC2 and 2 morecytosol, extracellular spaceOne of 2 routes

• trans-urocanateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by GATM, SLC7A5, SLC7A6, HALcytosol, extracellular spaceOne of 2 routes

• urateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. Peroxidase

  Catalysed by GATM, ARG1, MPOcytosol, nucleusOne of 4 routes

• 5-hydroxy-L-tryptophanReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by GATM, SLC7A6, TPH1, TPH2cytosol, extracellular space

• methylarsonous acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by GATM, GSS, GSTO1, GSTO2cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by GATM, SLC3A2, SLC7A6, SLC7A7 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 5 routes

• 3-oxopropanoic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC6A6, SLC6A14, ABATextracellular space, cytosol, mitochondrial matrix

• 3-phenylpyruvateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by GATM, SLC7A6, GOT2, TATcytosol, extracellular space, mitochondrial matrixOne of 6 routes

• fumaric acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by GATM, ASLcytosolOne of 4 routes

• O-octanoyl-(R)-carnitineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Carnitine Transport by Atb0
  3. solute carrier (L-carnitine)

  Catalysed by SLC6A14, SLC25A20extracellular space, cytosol, mitochondrial matrixOne of 7 routes

• CHEBI:18132Reach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and choline (cytosol to extracellular)
  3. ATP:choline phosphotransferase

  Catalysed by SLC5A7, CHKA, CHKBextracellular space, cytosol

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by GATM, GARTcytosol

• arsenic triglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by GATM, GSS, AS3MTcytosolOne of 3 routes

• 2-phenylethylamineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by GATM, SLC7A6, DDCcytosol, extracellular space

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by GATM, SLC7A6, PFAScytosol, extracellular space

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by GATM, AGXT2, GCLCcytosol

• S-methylmercury-L-cysteineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by GATM, SLC7A6, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 6 routes

• 2-aminoisobutanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by GATM, SLC7A6, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes