L-leucine

Two steps

• 3-disulfanyl-L-alanineReach from here →
  1. Transport of L-Cystine (L-Leucine antiport) by B0, +At Transporter
  2. L-cystine thiocysteine-lyase (deaminating

  Catalysed by SLC7A9, SLC3A1, CTHextracellular space, cytosol

• N-acetyl-L-cysteineReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by SLC7A9, SLC3A1, NAGSextracellular space, cytosol, mitochondrial matrix

• K(+)Reach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by SLC7A9, SLC3A1, SLC1A1extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

  Catalysed by BCAT2, BCKDHA, BCKDHBmitochondrial matrix

• Cys-tRNA(Cys)Reach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by SLC7A9, SLC3A1, CARS1extracellular space, cytosol

• Phe-tRNA(Phe)Reach from here →
  1. transport of leucine and phenylalanine (cytosol to extracellular)
  2. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by SLC7A5, FARSB, FARSAextracellular space, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by SLC7A5, GARS1extracellular space, cytosol

• Asp-tRNA(Asp)Reach from here →
  1. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  2. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by SLC7A13, DARS1inner side of the membrane, outer side of the membrane, cytosol

• Trp-tRNA(Trp)Reach from here →
  1. transport of leucine and tryptophan (cytosol to extracellular)
  2. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

  Catalysed by SLC7A5, WARS1extracellular space, cytosol

• Ile-tRNA(Ile)Reach from here →
  1. transport of isoleucine and leucine (cytosol to extracellular)
  2. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by SLC7A5, IARS1extracellular space, cytosol

• Gln-tRNA(Gln)Reach from here →
  1. transport of glutamine and leucine (cytosol to extracellular)
  2. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

  Catalysed by SLC7A5, QARS1extracellular space, cytosol

• isocyanateReach from here →
  1. transport of citrulline and leucine (cytosol to extracellular)
  2. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by LACC1extracellular space, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. transport of asparagine and leucine (cytosol to extracellular)
  2. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by SLC7A5, NARS1extracellular space, cytosol

• glycocholateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by SLC7A5, BAATextracellular space, cytosol, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by SLC7A9, SLC3A1, CTHextracellular space, cytosolOne of 2 routes

• CHEBI:30249Reach from here →
  1. transport of leucine and tryptophan (cytosol to extracellular)
  2. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by SLC7A5, IDO1, TDO2, IDO2extracellular space, cytosol

• formic acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)

  Catalysed by SLC6A14, SLC26A4extracellular space, cytosolOne of 2 routes

• anthranilic acidReach from here →
  1. Transport of L-Kynurenine
  2. L-Kynurenine hydrolase

  Catalysed by SLC43A1, KYNUextracellular space, cytosol

• malonic acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Malonate Transport, Antiport with Chloride

  Catalysed by SLC6A14extracellular space, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate
  2. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate

  Catalysed by PM20D1

• D-lysineReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by SLC7A6, SLC25A2extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• putrescineReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by SLC3A2, SLC7A6, SLC7A7extracellular space, cytosolOne of 4 routes

• D-arginineReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by SLC7A6, SLC25A2extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-carbamoyl-L-aspartateReach from here →
  1. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  2. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

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

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. transport of leucine and methionine (cytosol to extracellular)
  2. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC3A2, SLC7A5, IL4I1extracellular space, cytosol

• di-mu-sulfido-diiron(2+)Reach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. De novo synthesis of 2Fe2S

  Catalysed by SLC7A9, SLC3A1, NDUFAB1, FDXR and 5 moreextracellular space, cytosol, mitochondrial matrix

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. transport of isoleucine and leucine (cytosol to extracellular)
  2. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by SLC7A5, BCAT2, BCAT1extracellular space, cytosol

• D-cysteineReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 10 routes

• 5-aminolevulinateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by SLC7A5, ALAS1, ALAS2extracellular space, cytosol

• 3-methyl-2-oxovaleric acidReach from here →
  1. transport of isoleucine and leucine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A5, BCAT1extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. transport of leucine and tyrosine (cytosol to extracellular)
  2. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A5, GOT2, GOT1, TATextracellular space, cytosolOne of 3 routes

• glycochenodeoxycholateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by SLC7A5, BAATextracellular space, cytosol

• glycochenodeoxycholic acidReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by SLC7A5, BAATextracellular space, cytosol, peroxisomeOne of 3 routes

• glyoxylateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine

  Catalysed by BCAT2, AADATmitochondrial matrixOne of 12 routes

• 3-hydroxyisovaleric acidReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. 4-methyl-2-oxopentanoate decarboxylation

  Catalysed by BCAT2, ALOX12B, ALOXE3mitochondrial matrix, cytosol

• glycolithocholic acidReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by SLC7A5, BAATextracellular space, cytosolOne of 2 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by SLC7A5, GCATextracellular space, cytosol, mitochondrial matrix

• gamma-Glu-AlaReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. gamma-glutamyltransferase (alanine)

  Catalysed by SLC7A5, GGT1, GGT5, GGTLC2 and 3 moreextracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by SLC7A5, GLDC, GCSHextracellular space, cytosol, mitochondrial matrix

• taurineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC6A14, SLC6A6extracellular space, cytosolOne of 4 routes

• (R)-methylmalonyl-CoAReach from here →
  1. Acetylation of Leucine
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. Acetylation of Leucine
  2. (S)-methylmalonyl-CoA hydrolase

  No catalyst recordedmitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. Acetylation of Leucine
  2. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. (2E)-dodecenoyl-CoA formation

  No catalyst recordedmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

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

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 2 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. Acetylation of Leucine
  2. Production of Glutaryl Carnitine

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes