beta-alanine

Three steps

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

  Catalysed by CARNS1, NAA10, ACP2cytosol, lysosomeOne of 2 routes

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

  Catalysed by CARNS1, NAA10cytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by CARNS1, NAA10, ACAA1, MYO5Bcytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes

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

  Catalysed by CARNS1, NAA10, ACSM4, ACSM1 and 4 morecytosolOne of 11 routes

• dodecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 9 routes

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

  Catalysed by CARNS1, NAA10, CPT1A, CPT1C and 1 morecytosolOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 12 routes

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

  Catalysed by CARNS1, NAA10, SUCLG1, SUCLG2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

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

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

  Catalysed by CARNS1, NAA10, CROTcytosol, mitochondrial matrixOne of 20 routes

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

  Catalysed by CARNS1, NAA10cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 5 morecytosolOne of 15 routes

• prostaglandin H2Reach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by AGXT2, MOCOS, PTGS1, PTGS2One of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by AGXT2, MOCOS, TBXAS1, PTGS2 and 1 more

• sarcosineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by SLC6A6, SLC6A14, SLC6A9, PIPOXextracellular space, cytosolOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by SLC6A6, SLC6A14, MTO1extracellular space, cytosol

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by SLC6A6, SLC6A14, SLC6A9, SHMT1 and 1 moreextracellular space, cytosol

• 4-guanidinobutanoateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

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

• L-dopaReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by SLC6A6, SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

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

  Catalysed by SLC6A6, SLC6A14, CPT1A, CPT1C and 1 moreextracellular space, cytosolOne of 9 routes

• an N-acylglycineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by SLC6A6, SLC6A14, SLC6A9, GLYATL3 and 2 moreextracellular space, cytosol

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by SLC6A6, SLC6A14, GOT2, GOT1extracellular space, cytosolOne of 5 routes

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

  Catalysed by AGXT2mitochondrial matrix

• succinate 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 = succinate semialdehyde + L-glutamate

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

• sphing-4-enineReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by CARNS1, NAA10, ASAH1, ACER2 and 2 morecytosol, Golgi apparatusOne of 2 routes

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

  Catalysed by CARNS1, NAA10, SAT1, SAT2cytosolOne of 5 routes

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

  Catalysed by GADL1, CSAD, SLC25A12, SLC25A13mitochondrial matrix, cytosol

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SGSHextracellular space, cytosol

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, STSextracellular space, cytosol

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

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

• glutathioneReach 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 + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by SLC6A6, SLC6A14, SLC6A9, GSSextracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC6A6, SLC6A14, AASSextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• leukotriene C4Reach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by SLC6A6, SLC6A14, SLC6A9extracellular space, cytosol

• dimethylamineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by AGXT2, DDAH1cytosolOne of 2 routes

• IMPReach from here →
  1. L-aspartate + H(+) = beta-alanine + CO2
  2. IMP:L-aspartate ligase (GDP-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by GADL1, CSAD, ADSS2, ADSS1cytosol

• taurocyamineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. taurine + L-arginine = taurocyamine + L-ornithine

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

• L-cysteateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by SLC6A6, SLC6A14, GADL1, CSADextracellular space, cytosol

• L,L-cystathionineReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by CARNS1, NAA10, NAGScytosol, mitochondrial matrix

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

  Catalysed by SLC6A6, SLC6A14, GCLC, GCLMextracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by SLC6A6, SLC6A14extracellular space, cytosol, peroxisomeOne of 2 routes

• L-homocysteineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC6A6, SLC6A14, BHMT2extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Glutamine by Atb0 Transporter
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by SLC6A6, SLC6A14, CADextracellular space, cytosolOne of 6 routes

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

  Catalysed by SLC6A6, SLC6A14, SLC26A6, PAPSS1 and 1 moreextracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of betaine and chloride (cytosol to extracellular)
  3. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by SLC6A6, SLC6A14, SLC6A12, BHMTextracellular space, cytosol

• S-methyl-L-methionineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC6A6, SLC6A14, BHMT2extracellular space, cytosol

• N-acetylputrescineReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. acetyl-CoA:putrescine N-acetyltransferase

  Catalysed by GATM, ODC1, SAT1, SAT2cytosolOne of 2 routes

• 4-aminobutanalReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. putrescine + O2 + H2O = 4-aminobutanal + H2O2 + NH4(+)

  Catalysed by GATM, ODC1, AOC1cytosol

• L-glutamyl 5-phosphateReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate
  3. L-glutamate 5-semialdehyde + phosphate + NADP(+) = L-glutamyl 5-phosphate + NADPH + H(+)

  Catalysed by GATM, OAT, ALDH18A1