beta-alanine

Three steps

• glutathione disulfideReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  3. cystine to cysteine conversion

  Catalysed by AGXT2, SLC7A9, SLC3A1extracellular space, cytosol

• N(1)-acetylspermidineReach from here →
  1. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2

  Catalysed by GATM, ODC1, PAOXcytosol

• an acyl-CoAReach 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

• histamineReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. Nalpha-(beta-alanyl)-L-histidine hydrolase
  3. L-histidine carboxy-lyase (histamine-forming)

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

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

  Catalysed by AGXT2, KYNU, AADATOne of 2 routes

• L-thyroxineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and iodide (cytosol to extracellular)
  3. 3,3',5-triiodo-L-thyronine,iodide:acceptor oxidoreductase (iodinating)

  Catalysed by SLC6A6, SLC6A14, SLC26A4, DIO1 and 1 moreextracellular space, cytosolOne of 2 routes

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

  Catalysed by AGXT2, KYNU, KYAT3, AADATmitochondrial matrixOne of 13 routes

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

  Catalysed by AGXT2, KYNU, AADATOne of 2 routes

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. L-aspartate + H(+) = beta-alanine + CO2
  2. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by GADL1, CSAD, PAICS, ADSLcytosolOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by SLC6A6, SLC6A14, IL4I1extracellular space, cytosol

• (3Z)-dodecenoyl-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-oxomyrist-5-enoyl-CoA)

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

• S-sulfanyl-L-cysteineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by SLC6A6, SLC6A14, CARS2extracellular space, cytosol

• N-formyl-L-kynurenineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by SLC6A6, SLC6A14, IDO1, TDO2 and 1 moreextracellular space, cytosolOne of 2 routes

• (3S)-citramalyl-CoAReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by AGXT2, CLYBLmitochondrial matrix

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

  Catalysed by AGXT2, KYNU, AADAT

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by CARNS1, NAA10, SLC27A2, SLC27A5cytosol, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Glutamine by Atb0 Transporter
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

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

• phosphoenolpyruvateReach from here →
  1. L-aspartate + H(+) = beta-alanine + CO2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by GADL1, CSAD, GOT2, PCK1 and 1 moremitochondrial matrix

• 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. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

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

• L-methionine (S)-S-oxideReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC6A6, SLC6A14, MSRAextracellular space, cytosol

• L-methionine (R)-S-oxideReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC6A6, SLC6A14, MSRB3, MSRB1 and 1 moreextracellular space, cytosol

• deoxycholoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by CARNS1, NAA10, SLC27A2, SLC27A5cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by SLC6A6, SLC6A14, SLC6A9, GLYATL2extracellular space, cytosolOne of 2 routes

• methylamineReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

  Catalysed by AGXT2, DDAH1

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by SLC6A6, SLC6A14, PPCSextracellular space, cytosolOne of 4 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. L-aspartate + H(+) = beta-alanine + CO2
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  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 GADL1, CSAD, SLC7A13, PAHinner side of the membrane, outer side of the membrane

• pyridoxal 5'-phosphateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by AGXT2, SCLY, PNPOcytosol

• an L-alpha-amino acidReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

  Catalysed by AGXT2, KYNU, SLC7A11inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-iodo-L-tyrosineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. thyroid peroxidase (iodine)

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

• estrone 3-sulfateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

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

• N-benzoylglycineReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and glycine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by SLC6A6, SLC6A14, SLC6A9extracellular space, cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by CARNS1, NAA10, ACSM4, ACSM1 and 4 morecytosolOne of 7 routes

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

  Catalysed by CARNS1, NAA10, CPT1A, CPT1C and 1 morecytosolOne of 4 routes

• (9Z)-hexadecenoyl-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 (palmitolate)

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

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by AGXT2, HOGA1mitochondrial matrix

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

  Catalysed by CARNS1, NAA10, CPT1A, CPT1Bcytosol

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by SLC6A6, SLC6A14, CLTRN, SLC6A20 and 3 moreextracellular space, cytosolOne of 5 routes

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. transport of beta-alanine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by SLC6A6, SLC6A14, SLC26A6, SLC4A1extracellular space, cytosolOne of 2 routes

• CHEBI:63544Reach 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 (adrenic acid)

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

• hexacosanoyl-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 (cerotic acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes

• tetracosanoyl-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 (lignocerate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 10 routes

• docosanoyl-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 (behenic acid)

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

• a 2,3-saturated acyl-CoAReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by AGXT2, MOCOS, ACAD10

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach 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 (N-(omega)-hydroxyarginine-forming)

  Catalysed by SLC6A6, SLC6A14, NOS3, NOS1 and 1 moreextracellular space, cytosol

• xanthurenateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by AGXT2, KYNU, KYAT3, AADAT

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

  Catalysed by CARNS1, NAA10, CPT1A, CPT1C and 1 morecytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-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 (stearidonic acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 8 routes

• (8Z,11Z,14Z)-eicosatrienoateReach 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 reticulum

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by AGXT2, HOGA1mitochondrial matrix

• L-threonylcarbamoyladenylateReach from here →
  1. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

  Catalysed by AGXT2, SLC7A10, YRDCinner side of the membrane, outer side of the membrane