L-leucine

Two steps

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by SLC7A5, GARTextracellular space, cytosol

• L-cystine-bis-glycineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by SLC7A5, DPEP1, DPEP2extracellular space, cytosolOne of 2 routes

• glycyldehydrophenylalanineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by SLC7A5, DPEP1extracellular space, cytosol

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. transport of glutamine and leucine (cytosol to extracellular)
  2. 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 SLC7A5, PFASextracellular space, cytosol

• kinetensin-(1-8)Reach from here →
  1. Chymase
  2. kinetensin + H2O = kinetensin-(1-8) + L-leucine

  Catalysed by CMA1, ACE2extracellular space

• an N-(fatty acyl)-glycineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATL2extracellular space, cytosol

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATL2extracellular space, cytosol

• (7Z)-octadecenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• acetaldehydeReach from here →
  1. transport of leucine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by SLC7A5extracellular space, cytosol

• CHEBI:15345Reach from here →
  1. Acetylation of Leucine
  2. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by AACSmitochondrial matrix, cytosolOne of 2 routes

• cholineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)

  Catalysed by SLC6A14, SLC5A7extracellular space, cytosolOne of 2 routes

• pyruvateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by SLC7A5, AGXT2extracellular space, cytosol, mitochondrial matrixOne of 17 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. transport of leucine and tryptophan (cytosol to extracellular)
  2. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by SLC7A5, TPH1, TPH2extracellular space, cytosolOne of 3 routes

• CHEBI:15380Reach from here →
  1. Acetylation of Leucine
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by ALAS1, CPNE7mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. Acetylation of Leucine
  2. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Production of Tiglylcarnitine

  Catalysed by CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. Acetylation of Leucine
  2. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by GLYATmitochondrial matrix

• isovaleryl-CoAReach from here →
  1. Acetylation of Leucine
  2. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. Acetylation of Leucine
  2. propanoyl-CoA C-acyltransferase

  Catalysed by ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Palmitate:CoA ligase (AMP-forming)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Phenylacetate:CoA ligase

  No catalyst recordedmitochondrial matrix, cytosolOne of 3 routes

• propionyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Propanoate:CoA ligase (AMP-forming)

  Catalysed by ACSS3, ACSS2, ACSS1mitochondrial matrix, cytosolOne of 43 routes

• CHEBI:15570Reach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by SLC7A5, SLC3A2, SLC7A10extracellular space, cytosolOne of 5 routes

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

  Catalysed by SLC7A5, GNMTextracellular space, cytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of leucine and tryptophan (cytosol to extracellular)
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by SLC7A5, TPH1, TPH2extracellular space, cytosolOne of 2 routes

• CHEBI:15650Reach from here →
  1. Acetylation of Leucine
  2. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by ZDHHC11B, NAA10, ESCO2, ESCO1 and 28 moremitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by SLC7A6, DHPSextracellular space, cytosol

• CHEBI:15727Reach from here →
  1. transport of histidine and leucine (cytosol to extracellular)
  2. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by SLC7A6, CARNS1extracellular space, cytosolOne of 2 routes

• CHEBI:15746Reach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by SLC7A13, CADinner side of the membrane, outer side of the membrane, cytosol

• alpha-D-glucosamine 6-phosphateReach from here →
  1. transport of glutamine and leucine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by SLC7A5, GFPT2, GFPT1extracellular space, cytosol

• sulfateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)

  Catalysed by SLC6A14, SLC26A6extracellular space, cytosolOne of 3 routes

• ureaReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. L-Arginine amidinohydrolase

  Catalysed by SLC3A2, SLC7A6, SLC7A7, ARG1extracellular space, cytosolOne of 3 routes

• 11-cis-retinolReach from here →
  1. Acetylation of Leucine
  2. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 1 moremitochondrial matrix, cytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by SLC7A5, AGXT2extracellular space, cytosol, mitochondrial matrix

• iodideReach from here →
  1. transport of leucine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)

  Catalysed by SLC7A5, TPOextracellular space, cytosolOne of 3 routes

• oxaloacetateReach from here →
  1. Acetylation of Leucine
  2. ATP citrate lyase (citrate)

  Catalysed by ACLYmitochondrial matrix, cytosolOne of 6 routes

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

  Catalysed by SLC3A2, SLC7A6, SLC7A7, NOS3 and 2 moreextracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC7A5, SLC3A2, SLC7A10extracellular space, cytosolOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by SLC7A6, ACY1extracellular space, cytosol

• 2-phenylacetamideReach from here →
  1. transport of leucine and phenylalanine (cytosol to extracellular)
  2. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by SLC7A5, EPXextracellular space, cytosol

• anthranilateReach from here →
  1. Transport of L-Kynurenine
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by SLC43A1, KYNUextracellular space, cytosol

• CHEBI:16633Reach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SLC7A5, SCLYextracellular space, cytosol

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

  Catalysed by SLC3A2, SLC7A5, MARS1extracellular space, cytosol

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

  Catalysed by SLC3A2, SLC7A5, AADATextracellular space, cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. transport of leucine and phenylalanine (cytosol to extracellular)
  2. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by SLC7A5, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. transport of leucine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by SLC7A5, SDS, SDSLextracellular space, cytosol