L-ornithine

Three steps

• sphing-4-enineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ODC1, SAT1, SAT2, ASAH1 and 3 morecytosol, Golgi apparatusOne of 2 routes

• hypotaurineReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by SLC7A6, CDO1, GADL1, CSADextracellular space, cytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by SLC7A6, AGXT2, AGAextracellular space, cytosol

• N(1)-acetylspermineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

  Catalysed by ODC1, SRM, PAOXcytosol

• porphobilinogenReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by GATM, ALAS1, ALAS2, CPNE7 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• L,L-cystathionineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by ODC1, SAT1, SAT2, NAGScytosol, mitochondrial matrixOne of 2 routes

• phosphoethanolamineReach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. phosphoethanolamine + H2O = acetaldehyde + NH4(+) + phosphate

  Catalysed by SLC7A6, ETNPPLextracellular space, cytosol

• N,N-dimethylglycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by GATM, PIPOX, DMGDHcytosolOne of 2 routes

• glutathione disulfideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  3. cystine to cysteine conversion

  Catalysed by GATM, SLC7A9cytosol, inner side of the membrane, outer side of the membraneOne of 36 routes

• taurolithocholate 3-sulfateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and sulfotaurolithocholate (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• (S)-2-amino-6-oxohexanoateReach from here →
  1. transport of lysine and ornithine (cytosol to extracellular)
  2. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)
  3. L-saccharopine + NAD(+) + H2O = (S)-2-amino-6-oxohexanoate + L-glutamate + NADH + H(+)

  Catalysed by SLC7A5, SLC7A6, AASSextracellular space, cytosol, mitochondrial matrix

• an acyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ODC1, SAT1, SAT2, ASAH1 and 3 morecytosol, Golgi apparatusOne of 3 routes

• 3-aminopropanalReach from here →
  1. transport of lysine and ornithine (cytosol to extracellular)
  2. deoxyhypusine synthase (1,3-diaminopropane)
  3. primary-amine oxidase (1,3-diaminopropane)

  Catalysed by SLC7A5, SLC7A6, DHPS, AOC2 and 2 moreextracellular space, cytosol

• 5-oxo-L-prolineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. glutathione = L-cysteinylglycine + 5-oxo-L-proline

  Catalysed by GATM, GSS, CHAC2, CHAC1cytosolOne of 2 routes

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by SLC7A6, AGXT2, PAICSextracellular space, cytosolOne of 2 routes

• anserineReach from here →
  1. L-histidine(in) + L-ornithine(out) + H(+)(in) = L-histidine(out) + L-ornithine(in) + H(+)(out)
  2. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)
  3. carnosine + S-adenosyl-L-methionine = anserine + S-adenosyl-L-homocysteine + H(+)

  Catalysed by SLC25A2, CARNS1, CARNMT1inner side of the membrane, outer side of the membrane

• L-thyroxineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and thyroxine (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 4 routes

• kynurenateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-kynurenine + glyoxylate = kynurenate + glycine + H2O

  Catalysed by GATM, AADAT, KYAT3cytosolOne of 20 routes

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by GATM, SHMT1, ATICcytosol

• 5-(methylsulfanyl)-alpha-D-ribose 1-phosphateReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. S-methyl-5'-thioadenosine:phosphate S-methyl-5-thio-alpha-D-ribosyl-transferase

  Catalysed by ODC1, SRM, MTAPcytosolOne of 2 routes

• N(8)-acetylspermidineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. N8-acetylspermidine to spermidine conversion

  Catalysed by ODC1, SRMcytosolOne of 4 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by ODC1, SAT1, SAT2, HADHA and 2 morecytosol, mitochondrial matrixOne of 3 routes

• N(1),N(12)-diacetylspermineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2
  3. N(1),N(12)-diacetylspermine + O2 + H2O = 3-acetamidopropanal + N(1)-acetylspermidine + H2O2

  Catalysed by ODC1, PAOXcytosol

• N(tau)-methylhistamineReach from here →
  1. transport of histidine and ornithine (cytosol to extracellular)
  2. L-histidine carboxy-lyase (histamine-forming)
  3. S-Adenosyl-L-methionine:histamine N-tele-methyltransferase

  Catalysed by SLC7A5, SLC7A6, HDC, HNMTextracellular space, cytosolOne of 3 routes

• (3S)-citramalyl-CoAReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by SLC7A6, AGXT2, CLYBLextracellular space, cytosol, mitochondrial matrix

• L-2-aminoadipateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. 2-oxoadipate + L-kynurenine = L-2-aminoadipate + kynurenate + H2O

  Catalysed by SLC7A6, KYNU, AADATextracellular space, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 1 morecytosol, endoplasmic reticulumOne of 3 routes

• an N-acetyl-L-cysteine-S-conjugateReach from here →
  1. N2-Acetyl-L-ornithine amidohydrolase
  2. N2-Acetyl-L-ornithine amidohydrolase
  3. an N-acetyl-L-cysteine-S-conjugate + H2O = an S-substituted L-cysteine + acetate

  Catalysed by ACY1, ACY3cytosol

• deoxycholoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 1 morecytosolOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by GATM, GSS, SQORcytosol, mitochondrial matrixOne of 8 routes

• methylamineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. N(omega)-methyl-L-arginine(in) + L-arginine(out) = N(omega)-methyl-L-arginine(out) + L-arginine(in)
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

  Catalysed by GATM, SLC25A29, DDAH1cytosol, inner side of the membrane, outer side of the membrane

• monodehydro-L-ascorbate radicalReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. N-(9Z-octadecenoyl)glycine + 2 L-ascorbate + O2 = N-(9Z-octadecenoyl)-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by GATM, GLYATL3, GLYATL2, PAMcytosolOne of 5 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  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 GATM, AADAT, PAHcytosolOne of 4 routes

• pyridoxal 5'-phosphateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by SLC7A6, SCLY, PNPOextracellular space, cytosol

• 2-hydroxy-4-methylvaleric acidReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. 4-methyl-2-oxopentanoate reduction

  Catalysed by SLC7A6, BCAT2, SDR42E2, AKR1B15 and 16 moreextracellular space, cytosol, mitochondrial matrix

• an L-alpha-amino acidReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  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 SLC7A6, KYNU, SLC7A11extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. Putrescine:oxygen oxidoreductase (deaminating)
  3. 4-Aminobutyraldehyde:NAD+ oxidoreductase

  Catalysed by ODC1, AOC2, AOC1, AOC3 and 5 morecytosol, extracellular space, mitochondrial matrixOne of 11 routes

• dopamineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of dopamine and GSH (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 4 routes

• glycolithocholate 3-sulfateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA:amino acid N-acyltransferase (glycine)
  3. sulfotransferase (glycolithocholate)

  Catalysed by GATM, BAAT, SULT2A1cytosolOne of 3 routes

• a 2,3-dehydroacyl-CoAReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  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 SLC7A6, MOCOS, ACAD10extracellular space, cytosol

• prostaglandin E2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin E2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• decanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ODC1, SAT1, SAT2, ACSM4 and 5 morecytosolOne of 8 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Carnitine O-Palmitoyltransferase

  Catalysed by ODC1, SAT1, SAT2, CPT1A and 2 morecytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 11 routes

• Fe(II)-heme aReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

  Catalysed by SLC7A6, MOCOS, COX15extracellular space, cytosol

• (R)-4'-phosphopantetheineReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4-phosphopantothenoyl]-L-cysteine + H(+) = (R)-4'-phosphopantetheine + CO2

  Catalysed by SLC7A6, PPCS, PPCDCextracellular space, cytosol

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by GATM, AADAT, HOGA1cytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by ODC1, SAT1, SAT2, CPT1A and 1 morecytosol

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

  Catalysed by SLC7A6, SLC1A4, PYCR1, PYCR3 and 1 moreextracellular space, cytosolOne of 5 routes

• 2-deoxy-D-ribose 5-phosphateReach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. 2-deoxy-D-ribose 5-phosphate = D-glyceraldehyde 3-phosphate + acetaldehyde

  Catalysed by SLC7A6, DERAextracellular space, cytosol