guanidinoacetate

Three steps

• glycolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by GATM, DAO, GRHPRcytosol, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

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

• D-aspartateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. D-aspartate(in) + sulfate(out) = D-aspartate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-aspartateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)

  Catalysed by GATM, ASL, ASS1cytosolOne of 28 routes

• acetateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of acetate and formate (cytosol to extracellular)

  Catalysed by SLC26A4, SLC16A4, SLC16A5, SLC16A1extracellular space, cytosolOne of 2 routes

• CHEBI:30249Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by GATM, SLC7A6, IDO1, TDO2 and 1 morecytosol, extracellular space

• 3-acetamidopropanalReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Transport by Y+Lat-2 into Extracellular Space
  3. N1-acetylspermidine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by GATM, SLC3A2, SLC7A6, PAOX and 1 morecytosol, extracellular spaceOne of 2 routes

• oxalateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

  Catalysed by GATM, AADAT, HAO1cytosolOne of 3 routes

• propionic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of formate and propanoate (cytosol to extracellular)

  Catalysed by SLC26A4, SLC16A4, SLC16A5, SLC16A1extracellular space, cytosolOne of 2 routes

• citric acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• butyric acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of butyrate and formate (cytosol to extracellular)

  Catalysed by SLC26A4, SLC16A4, SLC16A5extracellular space, cytosolOne of 2 routes

• maleateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. (9Z)-octadecenoate + glycine = N-(9Z-octadecenoyl)glycine + H2O

  Catalysed by GATM, GLYATL3, GLYATL2, FAAH and 1 morecytosolOne of 3 routes

• an alcoholReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by GATM, GSS, CP, PRDX6cytosol

• isocitric acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• glutarateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycocholate(out) + glutarate(in) = glycocholate(in) + glutarate(out)

  Catalysed by GATM, BAAT, SLC22A24cytosol, peroxisome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (2R,3R)-tartrateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (9Z)-hexadecenoateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A14extracellular space, cytosol, endoplasmic reticulumOne of 6 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by GATM, GSScytosol, extracellular spaceOne of 5 routes

• D-lysineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by GATM, ARG1, SLC25A2cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-arginineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by GATM, ARG1, SLC25A2cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• 3-oxopropanoateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by SLC6A6, SLC6A14, AGXT2extracellular space, cytosol

• thiosulfateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30, SLC26A1 and 1 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by GATM, SLC7A6, IL4I1cytosol, extracellular space

• di-mu-sulfido-diiron(2+)Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by GATM, SLC7A6, NDUFAB1, FDXR and 5 morecytosol, extracellular space, mitochondrial matrix

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

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by GATM, SLC7A6, BCAT2, BCAT1cytosol, extracellular space

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

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

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

  Catalysed by GATM, SLC7A6, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 10 routes

• an acylcholineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and choline (cytosol to extracellular)
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by SLC5A7, BCHEextracellular space, cytosol

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

  Catalysed by GATM, SLC7A6, BCAT1cytosol, extracellular spaceOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by GATM, SLC7A6, GOT2, GOT1 and 1 morecytosol, extracellular spaceOne of 7 routes

• taurocholateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (GSH)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 6 routes

• taurodeoxycholateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by SLC6A6, BAATextracellular space, cytosolOne of 2 routes

• (S)-lactic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of formate and L-lactate (cytosol to extracellular)

  Catalysed by SLC26A4, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosolOne of 2 routes

• tert-butanolReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by GATM, GSS, CLIC2, GPX1 and 3 morecytosol

• gamma-Glu-AlaReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by GATM, SLC7A6, GGT1, GGT5 and 4 morecytosol, extracellular spaceOne of 2 routes

• 3,3',5-triiodo-L-thyronineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and triiodothyronine (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 6 routes

• (R)-methylmalonyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by GATM, BAAT, ALDH6A1cytosol, peroxisome, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by GATM, BAATcytosol, peroxisome, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by GATM, BAAT, ACP2cytosol, peroxisome, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by GATM, BAATcytosol, peroxisome, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by GATM, BAAT, ACAA1, MYO5Bcytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 7 routes

• benzoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by GATM, GLYATcytosol, mitochondrial matrix

• butanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by GATM, BAAT, ACSM4, ACSM1 and 4 morecytosol, peroxisomeOne of 11 routes