beta-alanine

Three steps

• CHEBI:15380Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by CARNS1, NAA10, ALAS1, CPNE7cytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by CARNS1, NAA10, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Production of Tiglylcarnitine

  Catalysed by CARNS1, NAA10, CPT1A, CPT1BcytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by CARNS1, NAA10, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by CARNS1, NAA10, GLYATcytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by CARNS1, NAA10, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by CARNS1, NAA10, ACAA1, SCP2 and 1 morecytosol, peroxisomeOne of 2 routes

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

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by CARNS1, NAA10, CPT1A, CPT1C and 1 morecytosolOne of 11 routes

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

  Catalysed by CARNS1, NAA10cytosolOne of 3 routes

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

  Catalysed by CARNS1, NAA10, ACSS3, ACSS2 and 1 morecytosolOne of 45 routes

• (S)-malateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of malate and sulfate (cytosol to mitochondria)

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SLC25A10extracellular space, cytosol, mitochondrial matrixOne of 11 routes

• CHEBI:15611Reach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

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

• 10-formyltetrahydrofolic acidReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by SLC6A6, SLC6A14, SLC26A4, MTHFD1extracellular space, cytosolOne of 2 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

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

• CHEBI:15650Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by CARNS1, NAA10, ZDHHC11B, ESCO2 and 29 morecytosolOne of 2 routes

• betaine aldehydeReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. choline dehydrogenase (choline)

  Catalysed by SLC6A6, SLC6A14, SLC5A7, CHDHextracellular space, cytosolOne of 3 routes

• trimethylenediamineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by SLC6A6, SLC6A14, DHPSextracellular space, cytosol

• CHEBI:15746Reach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by CARNS1, NAA10, SAT1, SAT2cytosolOne of 2 routes

• hexadecanoic 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

• CHEBI:15765Reach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by SLC6A6, SLC6A14, THextracellular space, cytosolOne of 5 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SLC25A14 and 2 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Glutamine by Atb0 Transporter
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by SLC6A6, SLC6A14, GFPT2, GFPT1extracellular space, cytosol

• N-acetyl-beta-D-glucosaminylamineReach from here →
  1. L-aspartate + H(+) = beta-alanine + CO2
  2. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by GADL1, CSAD, AGA

• thioredoxin dithiolReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-Alanine:tRNA(Ala) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by AGXT2, AARS1cytosolOne of 4 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)

  Catalysed by AGXT2, ABATmitochondrial matrixOne of 3 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC6A12, ABATextracellular space, cytosol, mitochondrial matrix

• 11-cis-retinolReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by CARNS1, NAA10, DGAT1, LRAT and 3 morecytosol

• cob(II)alaminReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 2 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

  Catalysed by AGXT2, MOCOS, MTRR

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

  Catalysed by SLC6A6, SLC6A14, SLC6A12, SLC22A5extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by SLC6A6, SLC6A14, NOS3, NOS1 and 1 moreextracellular space, cytosolOne of 3 routes

• selaneReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AGXT2, SCLYcytosolOne of 3 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC6A9, SLC3A2 and 1 moreextracellular space, cytosolOne of 4 routes

• 2-phenylacetamideReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by SLC6A6, SLC6A14, EPXextracellular space, cytosol

• anthranilateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by AGXT2, KYNU

• D-alanyl-D-alanineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. alanine racemase
  3. Formation of Alanylalanine

  Catalysed by AGXT2cytosol

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

  Catalysed by SLC6A6, SLC6A14, MARS1extracellular space, cytosol

• (S)-lactateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 2 moremitochondrial matrix

• CHEBI:16668Reach from here →
  1. L-aspartate + H(+) = beta-alanine + CO2
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by GADL1, CSAD, SLC25A12, SLC25A13 and 1 moremitochondrial matrix, cytosol

• quinolinic 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 Quinolinic Acid

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SLC22A6extracellular space, cytosolOne of 2 routes

• 3'-deoxy-3',4'-didehydro-CTPReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by AGXT2, MOCOS, RSAD2

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

  Catalysed by GATM, AGMAT, CA1, CA2

• pyridoxineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine-5'-phosphate phosphohydrolase

  Catalysed by AGXT2, SCLY, PHOSPHO2, PDXPcytosolOne of 2 routes

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

  Catalysed by SLC6A6, SLC6A14, AADATextracellular space, cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by SLC6A6, SLC6A14, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Threonine by Atb0 Transporter
  3. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by SLC6A6, SLC6A14, SDS, SDSLextracellular space, cytosolOne of 6 routes

• tryptamineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan decarboxy-lyase

  Catalysed by SLC6A6, SLC6A14, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Glutamine by Atb0 Transporter
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by SLC6A6, SLC6A14, KYAT1extracellular space, cytosolOne of 5 routes

• (S)-2-hydroxyglutarateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by AGXT2, MOCOS, L2HGDH