N(6)-(1,2-dicarboxyethyl)-AMP

Three steps

• a 1,4-benzosemiquinoneReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. a quinone + succinate = fumarate + a quinol
  3. 2 a quinone + NADPH + H(+) = 2 a 1,4-benzosemiquinone + NADP(+)

  Catalysed by ADSL, TNRC6B, SDHB, CRYZ and 3 morecytosol

• cholesterol sulfateReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Steryl-Sulfatase

  Catalysed by ADSL, TNRC6B, SLC25A10, STScytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• 5alpha-dihydrotestosterone sulfateReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. steroid sulfatase (5-alpha-dihydrotestosterone)

  Catalysed by ADSL, TNRC6B, SLC25A10, STScytosol, mitochondrial matrixOne of 3 routes

• acetoacetateReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  3. 4-fumarylacetoacetate fumarylhydrolase

  Catalysed by ADSL, TNRC6B, FAHcytosolOne of 2 routes

• an aryl sulfateReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. an aryl sulfate + H2O = a phenol + sulfate + H(+)

  Catalysed by ADSL, TNRC6B, SLC25A10, ARSL and 5 morecytosol, mitochondrial matrix

• 3-sulfinopyruvateReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase

  Catalysed by ADSS2, ADSS1, SLC25A12, SLC25A13 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• L-cystine-bis-glycineReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by ADSS2, ADSS1, SLC7A13, DPEP1 and 1 moreinner side of the membrane, outer side of the membrane

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by ADSS2, ADSS1, SLC7A13, PFASinner side of the membrane, outer side of the membrane

• kinetensinReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  3. Chymase

  Catalysed by ADSS2, ADSS1, SLC7A13, CMA1inner side of the membrane, outer side of the membrane, extracellular spaceOne of 3 routes

• (7Z)-octadecenoyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by ADSS2, ADSS1, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 3 routes

• CHEBI:15345Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by ADSS2, ADSS1, NAT8L, AACSmitochondrial matrix, cytosolOne of 2 routes

• cholineReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by ADSS2, ADSS1, NAT8L, CHATmitochondrial matrix, nucleus

• pyruvateReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate:NAD+ oxidoreductase (decarboxylating)

  Catalysed by ADSL, TNRC6B, FH, ME2 and 1 morecytosol, mitochondrial matrixOne of 27 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)

  Catalysed by ADSS2, ADSS1, SLC7A13, PAHinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• CHEBI:15380Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by ADSS2, ADSS1, NAT8L, ALAS1 and 1 moremitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by ADSS2, ADSS1, NAT8L, GLYAT and 2 moremitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Tiglylcarnitine

  Catalysed by ADSS2, ADSS1, NAT8L, CPT1A and 1 moremitochondrial matrix, cytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by ADSS2, ADSS1, NAT8L, HADHA and 2 moremitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by ADSS2, ADSS1, NAT8L, GLYATmitochondrial matrix

• isovaleryl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by ADSS2, ADSS1, NAT8L, GLYAT and 2 moremitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by ADSS2, ADSS1, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. propanoyl-CoA C-acyltransferase

  Catalysed by ADSS2, ADSS1, NAT8L, ACAA1 and 2 moremitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Palmitate:CoA ligase (AMP-forming)

  Catalysed by ADSS2, ADSS1, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by ADSS2, ADSS1, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Phenylacetate:CoA ligase

  Catalysed by ADSS2, ADSS1, NAT8Lmitochondrial matrix, cytosolOne of 3 routes

• propionyl-CoAReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by ADSS2, ADSS1, NAT8L, ACSS3 and 2 moremitochondrial matrix, cytosolOne of 43 routes

• CHEBI:15570Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. transport of D-alanine and glutamine (cytosol to extracellular)

  Catalysed by ADSS2, ADSS1, SLC7A13, SLC3A2 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 3 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  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 ADSS2, ADSS1, SLC7A13, PAHinner side of the membrane, outer side of the membraneOne of 3 routes

• CHEBI:15650Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by ADSS2, ADSS1, NAT8L, ZDHHC11B and 31 moremitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15699Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate:L-glutamine amido-ligase (AMP-forming)
  3. transport of asparagine and homoserine (cytosol to extracellular)

  Catalysed by ADSS2, ADSS1, ASNS, SLC7A5cytosol, extracellular spaceOne of 29 routes

• CHEBI:15727Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate 1-carboxy-lyase (beta-alanine-forming)
  3. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by ADSS2, ADSS1, GAD2, CARNS1cytosolOne of 2 routes

• CHEBI:15746Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by ADSS2, ADSS1, NAT8L, SAT1 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  3. solute carrier (L-dopa)

  Catalysed by ADSS2, ADSS1, SLC7A13, SLC3A2 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosol

• malonateReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)

  Catalysed by ADSL, TNRC6B, FH, SLC25A10cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by ADSS2, ADSS1, SLC7A13, GFPT2 and 1 moreinner side of the membrane, outer side of the membrane, cytosol

• beta-nicotinamide D-ribosideReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. a quinone + succinate = fumarate + a quinol
  3. 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamide + a quinone + H(+) = beta-nicotinamide D-riboside + a quinol

  Catalysed by ADSL, TNRC6B, SDHB, NQO2cytosol

• N-acetyl-beta-D-glucosaminylamineReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  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 ADSS2, ADSS1, AGA

• thioredoxin dithiolReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-Aspartate:tRNA(Asp) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by ADSS2, ADSS1, DARS1cytosolOne of 2 routes

• CHEBI:15971Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate:L-glutamine amido-ligase (AMP-forming)
  3. transport of asparagine and histidine (cytosol to extracellular)

  Catalysed by ADSS2, ADSS1, ASNS, SLC7A6cytosol, extracellular spaceOne of 18 routes

• CHEBI:16174Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. dGMP + inosine = 2'-deoxyguanosine + IMP
  3. dADP to dATP conversion

  Catalysed by ADSS2, ADSS1, NT5C2mitochondrial matrix

• 11-cis-retinolReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by ADSS2, ADSS1, NAT8L, DGAT1 and 4 moremitochondrial matrix, cytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by ADSS2, ADSS1, AGXT2mitochondrial matrix

• adenosineReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. IMP + H2O = inosine + phosphate
  3. transport of adenosine and inosine (cytosol to extracellular)

  Catalysed by ADSS2, ADSS1, NT5E, NT5C2 and 2 moreextracellular space, cytosolOne of 3 routes

• L-citrullineReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)

  Catalysed by ADSL, TNRC6B, ASL, ASS1cytosolOne of 18 routes

• L-arginineReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by ADSL, TNRC6B, ASLcytosolOne of 36 routes

• CHEBI:16523Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. transport of D-serine and glutamine (cytosol to extracellular)

  Catalysed by ADSS2, ADSS1, SLC7A13, SLC3A2 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• 2-phenylacetamideReach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by ADSS2, ADSS1, SLC7A13, EPXinner side of the membrane, outer side of the membrane, cytosol

• CHEBI:16633Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by ADSS2, ADSS1, AGXT2, SCLYcytosol

• Met-tRNA(Met)Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by ADSS2, ADSS1, SLC7A13, MARS1inner side of the membrane, outer side of the membrane, cytosol

• CHEBI:16668Reach from here →
  1. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by ADSS2, ADSS1, SLC25A12, SLC25A13 and 2 moremitochondrial matrix, cytosol