guanidinoacetate

Three steps

• dodecanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 10 routes

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

  Catalysed by GATM, BAAT, CPT1A, CPT1C and 1 morecytosol, peroxisomeOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by GATM, GLYATL3cytosol

• eicosanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 12 routes

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

  Catalysed by GATM, BAAT, SUCLG1, SUCLG2 and 1 morecytosol, peroxisome, mitochondrial matrixOne of 2 routes

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

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 14 routes

• tetradecanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 6 morecytosol, peroxisomeOne of 12 routes

• octanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by GATM, BAAT, CROTcytosol, peroxisome, mitochondrial matrixOne of 21 routes

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

  Catalysed by GATM, BAATcytosol, peroxisomeOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by GATM, BAAT, SLC27A2, ACSL4 and 5 morecytosol, peroxisomeOne of 15 routes

• prostaglandin E1Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin H2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin D2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by GATM, GSS, GPX5, GPX1 and 7 morecytosolOne of 2 routes

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

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

• 7,8-dihydrofolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase

  Catalysed by GATM, SHMT1, DHFR, DHFR2cytosolOne of 4 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by GATM, SHMT1, FTCDcytosolOne of 2 routes

• leukotriene A4Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 3 routes

• (R)-S-lactoylglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by GATM, GSS, GLO1, GLOD4cytosolOne of 3 routes

• carnosineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-histidine(out) + L-arginine(in) + Na(+)(out) = L-histidine(in) + L-arginine(out) + Na(+)(in)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by GATM, SLC7A6, CARNS1cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-oxoadipateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

  Catalysed by GATM, AADATcytosol

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

  Catalysed by GATM, SLC7A6, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 3 routes

• O-acetyl-(R)-carnitineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Carnitine Transport by Atb0
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by SLC6A14, CPT1A, CPT1C, CPT1Bextracellular space, cytosolOne of 9 routes

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

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

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by GATM, SLC7A6, GOT2, GOT1cytosol, extracellular spaceOne of 3 routes

• S-formylglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by GATM, GSS, ADH1C, ADH1A and 4 morecytosolOne of 2 routes

• succinate semialdehydeReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate

  Catalysed by SLC6A12, ABATextracellular space, cytosol

• sphing-4-enineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by GATM, BAAT, ASAH1, ACER2 and 2 morecytosol, peroxisome, Golgi apparatusOne of 2 routes

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

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

• spermidineReach 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 7 routes

• N-sulfo-D-glucosamineReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by SLC26A6, SGSHextracellular space, cytosol

• dehydroepiandrosterone 3-sulfateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by SLC26A6, STSextracellular space, cytosol

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

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

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

  Catalysed by GATM, SLC7A6, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by GATM, SLC3A2, SLC7A5, SLC7A6 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 2 routes

• L-kynurenineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

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

• dimethylamineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by GATM, SLC25A2, DDAH1cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-glutamate 5-semialdehydeReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by GATM, ARG1, OATcytosol

• L-cysteateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by SLC6A6, GADL1, CSADextracellular space, cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by GATM, SLC7A6, KYNUcytosol, extracellular space

• porphobilinogenReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by GATM, ALAS1, ALAS2, CPNE7 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• L,L-cystathionineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

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

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

  Catalysed by GATM, SLC7A6, GCLC, GCLMcytosol, extracellular spaceOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by GATM, SLC3A2, SLC7A5, SLC7A6cytosol, extracellular space, peroxisome

• L-homocysteineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by GATM, SLC7A6, BHMT2cytosol, extracellular space

• carbamoyl phosphateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Carbamoyl-phosphate:L-ornithine carbamoyltransferase

  Catalysed by GATM, NOS3, NOS1, NOS2 and 1 morecytosol, mitochondrial matrixOne of 4 routes

• adenosine 5'-phosphosulfateReach from here →
  1. Transport of Guanidinoacetic Acid
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. ATP:sulfate adenylyltransferase

  Catalysed by SLC26A6, PAPSS1, PAPSS2extracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by GATM, PIPOX, DMGDHcytosolOne of 2 routes