guanidinoacetate

Three steps

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by GATM, SLC7A6, PFAScytosol, extracellular space

• kinetensinReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by GATM, SLC7A6, CMA1cytosol, extracellular spaceOne of 3 routes

• (7Z)-octadecenoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by GATM, BAAT, CPT1A, CPT1C and 1 morecytosol, peroxisomeOne of 3 routes

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

  Catalysed by GATM, SLC7A6cytosol, extracellular space

• CHEBI:15345Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by GATM, BAAT, AACScytosol, peroxisomeOne of 2 routes

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

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 5 routes

• pyruvateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by GATM, SLC7A6, AGXT2cytosol, extracellular space, mitochondrial matrixOne of 23 routes

• all-trans-retinoic acidReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of formate and retinoate (cytosol to extracellular)

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

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by GATM, SLC7A6, TPH1, TPH2cytosol, extracellular spaceOne of 3 routes

• CHEBI:15380Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by GATM, BAAT, ALAS1, CPNE7cytosol, peroxisome, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by GATM, BAAT, GLYAT, GLYATL2 and 1 morecytosol, peroxisome, mitochondrial matrixOne of 3 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of Tiglylcarnitine

  Catalysed by GATM, BAAT, CPT1A, CPT1Bcytosol, peroxisomeOne of 2 routes

• isobutyryl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by GATM, BAAT, HADHA, ACAA2 and 1 morecytosol, peroxisome, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by GATM, BAAT, GLYATcytosol, peroxisome, mitochondrial matrix

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

  Catalysed by GATM, BAAT, GLYAT, GLYATL2 and 1 morecytosol, peroxisome, mitochondrial matrixOne of 3 routes

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

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 11 routes

• CHEBI:15519Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. propanoyl-CoA C-acyltransferase

  Catalysed by GATM, BAAT, ACAA1, SCP2 and 1 morecytosol, peroxisomeOne of 2 routes

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

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 11 routes

• oleoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by GATM, BAAT, CPT1A, CPT1C and 1 morecytosol, peroxisomeOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Phenylacetate:CoA ligase

  Catalysed by GATM, BAATcytosol, peroxisomeOne of 4 routes

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

  Catalysed by GATM, BAAT, ACSS3, ACSS2 and 1 morecytosol, peroxisomeOne of 45 routes

• (S)-malateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]

  Catalysed by GATM, DAO, CLYBLcytosol, peroxisome, mitochondrial matrixOne of 6 routes

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by GATM, SHMT1, SHMT2cytosolOne of 2 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by GATM, SHMT1, MTHFD1cytosolOne of 5 routes

• 5-methyltetrahydrofolic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. histamine N-methyltransferase (SAM)

  Catalysed by GATM, SHMT1, HNMTcytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach 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 spaceOne of 3 routes

• CHEBI:15650Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by GATM, BAAT, ZDHHC11B, NAA10 and 30 morecytosol, peroxisomeOne of 6 routes

• betaine aldehydeReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and choline (cytosol to extracellular)
  3. choline dehydrogenase (choline)

  Catalysed by SLC5A7, CHDHextracellular space, cytosolOne of 3 routes

• trimethylenediamineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by GATM, SLC3A2, SLC7A5, SLC7A6 and 1 morecytosol, extracellular space

• CHEBI:15727Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and histidine (cytosol to extracellular)
  3. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by GATM, SLC7A5, SLC7A6, CARNS1cytosol, extracellular spaceOne of 3 routes

• CHEBI:15746Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by GATM, BAAT, SAT1, SAT2cytosol, peroxisomeOne of 2 routes

• hexadecanoic 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

• CHEBI:15765Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by GATM, SLC7A6, THcytosol, extracellular spaceOne of 5 routes

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

  Catalysed by SLC26A6, SLC25A14, SLC25A30, SLC25A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• bromideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by GATM, GSS, MGST3, GSTA4 and 15 morecytosolOne of 2 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by GATM, SLC7A6, GFPT2, GFPT1cytosol, extracellular space

• thioredoxin dithiolReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:tRNA(Gly) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by GATM, GARS1cytosolOne of 2 routes

• a halide anionReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by GATM, GSS, GSTT4, GSTA4 and 21 morecytosol

• succinic semialdehydeReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate:2-oxoglutarate aminotransferase

  Catalysed by SLC6A12, ABATextracellular space, cytosol, mitochondrial matrix

• nitriteReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. 4 nitric oxide + O2 + 2 H2O = 4 nitrite + 4 H(+)

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

• 11-cis-retinolReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by GATM, BAAT, DGAT1, LRAT and 3 morecytosol, peroxisome

• (R)-3-aminoisobutyric acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by GATM, SLC7A6, AGXT2cytosol, extracellular space, mitochondrial matrix

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

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

• (R)-carnitineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of betaine and chloride (cytosol to extracellular)
  3. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)

  Catalysed by SLC6A12, SLC22A5extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• oxaloacetateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. ATP citrate lyase (citrate)

  Catalysed by GATM, BAAT, ACLYcytosol, peroxisome

• N(2)-acetyl-L-ornithineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by GATM, ARG1, ACY1cytosol

• 2-phenylacetamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by GATM, SLC7A6, EPXcytosol, extracellular space

• D-alanyl-D-alanineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of D-alanine and glycine (cytosol to extracellular)
  3. Formation of Alanylalanine

  Catalysed by GATM, SLC3A2, SLC7A10cytosol, extracellular space

• CHEBI:16633Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by GATM, SLC7A6, SCLYcytosol, extracellular space

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

  Catalysed by GATM, SLC7A6, MARS1cytosol, extracellular space