beta-alanine

Three steps

• 3-fumarylpyruvateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1mitochondrial matrix

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

  Catalysed by SLC6A6, SLC6A14, SLC6A9, GSSextracellular space, cytosolOne of 3 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC6A9, DAOextracellular space, cytosol, peroxisome

• trans-1,2-dithiane-4,5-diolReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by AGXT2, SCLYcytosol

• creatineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of Guanidinoacetic Acid
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by SLC6A6, SLC6A14, GAMTextracellular space, cytosolOne of 2 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of (R)-3-hydroxybutanoate and formate (cytosol to extracellular)

  Catalysed by SLC6A6, SLC6A14, SLC26A4, SLC16A4 and 3 moreextracellular space, cytosolOne of 4 routes

• taurolithocholateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. bile acid-CoA:amino acid N-acyltransferase (lithocholate)

  Catalysed by SLC6A6, SLC6A14, BAATextracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by AGXT2, AGXTmitochondrial matrixOne of 2 routes

• anilineReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by CARNS1, NAA10, NAT2, NAT1cytosol

• aceneuramateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by AGXT2, NPLmitochondrial matrix

• CHEBI:17312Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by CARNS1, NAA10cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by AGXT2, TYW1B, TYW1mitochondrial matrixOne of 5 routes

• linoleic acidReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A6, SLC6A14extracellular space, cytosol, endoplasmic reticulumOne of 2 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SLC25A14 and 1 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• D-aspartic acidReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by SLC6A6, SLC6A14, SLC12A2, SLC12A1 and 5 moreextracellular space, cytosol

• CHEBI:17380Reach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by SLC6A6, SLC6A14, SLC7A5extracellular space, cytosolOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Proline by the Apical Imino Amino Acid Transporters
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by SLC6A6, SLC6A14, CLTRN, SLC6A20 and 1 moreextracellular space, cytosolOne of 5 routes

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

  Catalysed by SLC6A6, SLC6A14extracellular space, cytosol, mitochondrial matrix

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by SLC6A6, SLC6A14, CPT2, CPT1Aextracellular space, cytosol, peroxisomeOne of 10 routes

• S-methyl-5'-thioadenosineReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by GATM, ODC1, SRMcytosolOne of 2 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC6A9, ALAS1 and 2 moreextracellular space, cytosol, mitochondrial matrix

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

  Catalysed by SLC6A6, SLC6A14, SLC26A4, TPOextracellular space, cytosolOne of 2 routes

• indole-3-pyruvateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by SLC6A6, SLC6A14, IL4I1extracellular space, cytosolOne of 2 routes

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by AGXT2, MOCOS, DIO1

• 3,3'-diiodo-L-thyronineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3One of 2 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, STSextracellular space, cytosol, Golgi apparatusOne of 4 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by CARNS1, NAA10, HADHA, ACADVL and 1 morecytosol, mitochondrial matrix

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

  Catalysed by SLC6A6, SLC6A14, SLC6A12, BHMT and 1 moreextracellular space, cytosol

• CHEBI:17769Reach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by GATM, ODC1, AOC2, AOC1 and 1 morecytosol, extracellular spaceOne of 2 routes

• trans-urocanateReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. Nalpha-(beta-alanyl)-L-histidine hydrolase
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by CARNS1, CNDP1, CNDP2, HALcytosolOne of 2 routes

• urateReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. 3-guanidinopropanoate + H2O = urea + beta-alanine
  3. Peroxidase

  Catalysed by GATM, AGMAT, MPOnucleusOne of 3 routes

• 5-hydroxy-L-tryptophanReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  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 SLC6A6, SLC6A14, TPH1, TPH2extracellular space, cytosol

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

  Catalysed by SLC6A6, SLC6A14, BCAT2extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• CHEBI:17927Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by CARNS1, NAA10cytosolOne of 2 routes

• 3-phenylpyruvateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by SLC6A6, SLC6A14, GOT2, TATextracellular space, cytosol, mitochondrial matrixOne of 5 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SLC25A10extracellular space, cytosol, mitochondrial matrixOne of 8 routes

• dTDPReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate kinase (dTDP)

  Catalysed by AGXT2, PKM, PKLRmitochondrial matrix, cytosol

• O-octanoyl-(R)-carnitineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. solute carrier (L-carnitine)

  Catalysed by SLC6A6, SLC6A14, SLC25A20extracellular space, cytosol, mitochondrial matrixOne of 7 routes

• CHEBI:18132Reach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. ATP:choline phosphotransferase

  Catalysed by SLC6A6, SLC6A14, SLC5A7, CHKA and 1 moreextracellular space, cytosol

• CHEBI:18183Reach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by AGXT2, GGT1, GGT5, GGTLC2 and 5 moreextracellular space, cytosol

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

  Catalysed by SLC6A6, SLC6A14, SLC6A9, GARTextracellular space, cytosol

• 2-phenylethylamineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by SLC6A6, SLC6A14, DDCextracellular space, cytosol

• CHEBI:18405Reach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by AGXT2, SCLY, PNPOcytosolOne of 3 routes

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Glutamine by Atb0 Transporter
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by SLC6A6, SLC6A14, PFASextracellular space, cytosol

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by AGXT2, GCLC

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

  Catalysed by SLC6A6, SLC6A14, SLC6A9, GSSextracellular space, cytosol

• S-methylmercury-L-cysteineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  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 SLC6A6, SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• D-isoleucineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membrane

• Xaa-L-Pro dipeptideReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)
  3. Xaa-L-Pro dipeptide + H2O = an L-alpha-amino acid + L-proline

  Catalysed by AGXT2, SLC1A4, PEPDinner side of the membrane, outer side of the membrane

• biocytinReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine(out) + L-lysine(in) = L-ornithine(in) + L-lysine(out)
  3. biocytin + H2O = biotin + L-lysine

  Catalysed by GATM, SLC25A2, SLC25A15, BTDinner side of the membrane, outer side of the membrane