N-carbamoyl-beta-alanine

Three steps

• (R)-carnitineReach from here →
  1. Transport of 3-Ureidopropionate
  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

• L-citrullineReach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. transport of alanine and citrulline (cytosol to extracellular)

  Catalysed by UPB1, AGXT2, SLC7A6cytosol, extracellular spaceOne of 48 routes

• oxaloacetateReach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. L-aspartate + H(+) = beta-alanine + CO2
  3. L-Aspartate:2-oxoglutarate aminotransferase

  Catalysed by UPB1, GADL1, CSAD, GOT2cytosol, mitochondrial matrixOne of 5 routes

• nitric oxideReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by SLC6A14, NOS3, NOS1, NOS2extracellular space, cytosolOne of 2 routes

• CHEBI:16523Reach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and glycine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by SLC6A9, SLC6A14, SLC3A2, SLC7A10extracellular space, cytosolOne of 4 routes

• protein polypeptide chainReach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. PROTEIN_BS

  Catalysed by UPB1, AGXT2cytosolOne of 18 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by UPB1, GATM, ACY1cytosol

• 2-phenylacetamideReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by SLC6A14, EPXextracellular space, cytosol

• CHEBI:16633Reach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by UPB1, AGXT2, SCLYcytosol

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

  Catalysed by SLC6A14, MARS1extracellular space, cytosol

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

  Catalysed by SLC26A6, SLC22A6extracellular space, cytosolOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by SLC6A14, AADATextracellular space, cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by SLC6A14, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Threonine by Atb0 Transporter
  3. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by SLC6A14, SDS, SDSLextracellular space, cytosol

• tryptamineReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan decarboxy-lyase

  Catalysed by SLC6A14, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Glutamine by Atb0 Transporter
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by SLC6A14, KYAT1extracellular space, cytosolOne of 3 routes

• CHEBI:16856Reach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and glycine (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by SLC6A9, SLC6A14, GSSextracellular space, cytosolOne of 2 routes

• glyoxylic acidReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and glycine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC6A9, SLC6A14, DAOextracellular space, cytosol, peroxisome

• creatineReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of Guanidinoacetic Acid
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by GAMTextracellular space, cytosolOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. L-aspartate + H(+) = beta-alanine + CO2
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by UPB1, GADL1, CSAD, NAT8Lcytosol, mitochondrial matrixOne of 2 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. Transport of 3-Ureidopropionate
  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. Transport of 3-Ureidopropionate
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. bile acid-CoA:amino acid N-acyltransferase (lithocholate)

  Catalysed by SLC6A6, BAATextracellular space, cytosolOne of 2 routes

• L-glutamic 5-semialdehydeReach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by UPB1, GATM, OATcytosol, mitochondrial matrix

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

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

• sulfiteReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)

  Catalysed by SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• D-aspartic acidReach from here →
  1. Transport of 3-Ureidopropionate
  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. Transport of 3-Ureidopropionate
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by SLC6A14, SLC7A5extracellular space, cytosolOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Proline by the Apical Imino Amino Acid Transporters
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by CLTRN, SLC6A20, PRODH2extracellular space, cytosolOne of 2 routes

• agmatineReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by SLC6A14extracellular space, cytosol, mitochondrial matrix

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. Transport of 3-Ureidopropionate
  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. 3-ureidopropanoate amidohydrolase
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by UPB1, AGXT2, MOCOScytosolOne of 6 routes

• 5-aminolevulinic acidReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and glycine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by SLC6A9, SLC6A14, ALAS1, ALAS2 and 1 moreextracellular space, cytosol, mitochondrial matrix

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

  Catalysed by SLC26A4, TPOextracellular space, cytosolOne of 2 routes

• indole-3-pyruvateReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by SLC6A14, IL4I1extracellular space, cytosolOne of 2 routes

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

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

• CHEBI:17724Reach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of betaine and chloride (cytosol to extracellular)
  3. Trimethylaminoacetate:L-homosysteine S-methyltransferase

  Catalysed by SLC6A12, BHMT, BHMT2extracellular space, cytosol

• Ala-tRNA(Ala)Reach from here →
  1. 3-ureidopropanoate amidohydrolase
  2. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by UPB1, AGXT2, AARS1cytosol

• trans-urocanateReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by SLC6A14, HALextracellular space, cytosol

• 5-hydroxy-L-tryptophanReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Tryptophan by Atb0 Transporter
  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 SLC6A14, TPH1, TPH2extracellular space, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by SLC6A14, BCAT2extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• CHEBI:17927Reach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of arginine and chloride (cytosol to extracellular)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by SLC6A14, SLC3A2, SLC7A6extracellular space, cytosol

• 3-phenylpyruvateReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by SLC6A14, GOT2, TATextracellular space, cytosol, mitochondrial matrixOne of 5 routes

• fumaric acidReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of fumarate and sulfate (cytosol to mitochondria)

  Catalysed by SLC26A6, SLC25A10extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• O-octanoyl-(R)-carnitineReach from here →
  1. Transport of 3-Ureidopropionate
  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 3-Ureidopropionate
  2. transport of chloride and choline (cytosol to extracellular)
  3. ATP:choline phosphotransferase

  Catalysed by SLC5A7, CHKA, CHKBextracellular space, cytosol

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and glycine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by SLC6A9, SLC6A14, GARTextracellular space, cytosol

• 2-phenylethylamineReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by SLC6A14, DDCextracellular space, cytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Glutamine by Atb0 Transporter
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by SLC6A14, PFASextracellular space, cytosol

• ophthalmateReach from here →
  1. Transport of 3-Ureidopropionate
  2. transport of chloride and glycine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by SLC6A9, SLC6A14, GSSextracellular space, cytosol

• S-methylmercury-L-cysteineReach from here →
  1. Transport of 3-Ureidopropionate
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. S-methylmercury-L-cysteine(in) + L-phenylalanine(out) = S-methylmercury-L-cysteine(out) + L-phenylalanine(in)

  Catalysed by SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes