3-guanidinopropanoate

Three steps

• 5-amino-2-oxopentanoateReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by AGMAT, GATM, AGXT2One of 2 routes

• 4-aminobutanoateReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by AGMAT, SLC6A6, SLC6A14, SLC6A12extracellular space, cytosolOne of 4 routes

• L-prolineReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by AGMAT, AGXT2, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. L-aspartate + H(+) = beta-alanine + CO2
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by AGMAT, GADL1, CSAD, SLC25A12 and 1 moremitochondrial matrix, cytosolOne of 4 routes

• L-cysteinylglycineReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. gamma-glutamyltransferase (alanine)

  Catalysed by AGMAT, AGXT2, GGTLC3, GGT1 and 4 morecytosolOne of 2 routes

• Mo-molybdopterinReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by AGMAT, AGXT2, MOCOS

• CHEBI:730073Reach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. Peroxidase
  3. Peroxidase

  Catalysed by AGMAT, MPOnucleusOne of 3 routes

• (2S)-2-aminobutanoateReach from here →
  1. 3-guanidinopropanoate + H2O = urea + beta-alanine
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by AGMAT, AGXT2