L-leucine

Three steps

• (2E,4Z,7Z)-decatrienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial
  3. Fatty Acid Beta Oxidation (C10:2->C10:3), Mitochondrial

  Catalysed by ECHS1, ACAA2, HADH, ACADMmitochondrial matrixOne of 3 routes

• 3-(glutathion-S-yl)-4-hydroxynonanalReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (E)-4-hydroxynon-2-enal + glutathione = 3-(glutathion-S-yl)-4-hydroxynonanal

  Catalysed by SLC7A5, GSS, GSTP1extracellular space, cytosol

• glycoursodeoxycholate 3-sulfateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. glycine to glycoursodeoxycholate conversion
  3. glycoursodeoxycholate + 3'-phosphoadenylyl sulfate = glycoursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLC7A5, SULT2A1extracellular space, cytosol

• glycochenodeoxycholate 3-sulfateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)
  3. glycochenodeoxycholate + 3'-phosphoadenylyl sulfate = glycochenodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLC7A5, BAAT, SULT2A1extracellular space, cytosol

• deoxycholateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. Glycodeoxycholate amidohydrolase
  3. Glycodeoxycholate amidohydrolase

  Catalysed by SLC7A5extracellular space, cytosol

• docosanoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• a quinolReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol

  Catalysed by SLC7A5, GSS, SQORextracellular space, cytosolOne of 4 routes

• octadecanoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Stearoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• octanoateReach from here →
  1. Acetylation of Leucine
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by CROT, ACOT7mitochondrial matrix, cytosolOne of 2 routes

• imidazole-4-acetaldehydeReach from here →
  1. transport of histidine and leucine (cytosol to extracellular)
  2. L-histidine carboxy-lyase (histamine-forming)
  3. amine oxidase copper containing (histamine)

  Catalysed by SLC7A6, HDC, AOC2, AOC1 and 1 moreextracellular space, cytosolOne of 2 routes

• alpha-linolenic acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A14extracellular space, cytosol, endoplasmic reticulumOne of 2 routes

• folic acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Folate Transport by Oats

  Catalysed by SLC6A14, SLC26A6, SLC22A8, SLC22A7extracellular space, cytosolOne of 2 routes

• selenomethionineReach from here →
  1. transport of leucine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by SLC7A5, SDS, SDSL, CTHextracellular space, cytosol

• decanoateReach from here →
  1. Acetylation of Leucine
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. Decanoyl-CoA hydrolase

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 3 moremitochondrial matrix, cytosolOne of 2 routes

• CHEBI:27760Reach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by SLC7A5, SCLY, CTHextracellular space, cytosolOne of 2 routes

• 3,4-dihydroxyphenylacetaldehydeReach from here →
  1. solute carrier (L-dopa)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. monoamine oxidase (dopamine)

  Catalysed by SLC3A2, SLC7A5, DDC, AOC2 and 4 moreextracellular space, cytosolOne of 2 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• 5(S)-HETEReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by SLC7A5, GSS, GPX5, GPX1 and 7 moreextracellular space, cytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• (2-hydroxyphenyl)acetic acidReach from here →
  1. transport of leucine and phenylalanine (cytosol to extracellular)
  2. Phenylalanine Transaminase, Mitochondrial
  3. Phenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by SLC7A5, GOT2, TAT, HPDextracellular space, cytosol, mitochondrial matrix

• 3-mercaptolactic acidReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by SLC7A9, SLC3A1, GOT2, GOT1 and 4 moreextracellular space, cytosol

• 2-oxopentanoateReach from here →
  1. Transport of L-Kynurenine
  2. 2-oxopentanoate + L-kynurenine = L-2-aminopentanoate + kynurenate + H2O
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by SLC43A1, AADAT, AGXT2extracellular space, cytosol

• O-decanoyl-(R)-carnitineReach from here →
  1. Acetylation of Leucine
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 4 moremitochondrial matrix, cytosol

• a dicarboxylateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  3. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)

  Catalysed by BCAT2, AADAT, SLC22A8mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 6 routes

• L-ascorbic acidReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. L-dehydroascorbate + 2 glutathione = glutathione disulfide + L-ascorbate

  Catalysed by SLC7A5, GSS, CLIC1, GSTO1 and 1 moreextracellular space, cytosol

• Ca(2+)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)
  3. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)

  Catalysed by SLC7A9, SLC3A1, SLC1A1, SLC24A1 and 4 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• hypoioditeReach from here →
  1. transport of leucine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by SLC7A5, TPO, MPO, LPOextracellular space, cytosol

• a thiolReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

  Catalysed by SLC7A9, SLC3A1, CTH, SUMF1extracellular space, cytosol

• hydrogenselenideReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)

  Catalysed by SLC7A5, MOCOS, SCLYextracellular space, cytosol

• phosphocholineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by SLC6A14, SLC5A7, PHOSPHO1extracellular space, cytosolOne of 2 routes

• cholateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (cholate)

  Catalysed by SLC7A5, GSS, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 5 routes

• glycolateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. glycolate + NADP(+) = glyoxylate + NADPH + H(+)

  Catalysed by BCAT2, AADAT, GRHPRmitochondrial matrixOne of 3 routes

• D-aspartateReach 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)
  3. D-aspartate(out) + K(+)(in) + 3 Na(+)(out) + H(+)(out) = D-aspartate(in) + K(+)(out) + 3 Na(+)(in) + H(+)(in)

  Catalysed by SLC7A9, SLC3A1, SLC1A1, SLC1A7 and 3 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

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

  Catalysed by SLC7A5, AGXT2, SLC16A4, SLC16A5 and 3 moreextracellular space, cytosol, mitochondrial matrixOne of 11 routes

• (9Z,12Z)-octadecadienoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosol

• 3-acetamidopropanalReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Transport by Y+Lat-2 into Extracellular Space
  3. N1-acetylspermidine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by SLC3A2, SLC7A6, SLC7A7, PAOX and 1 moreextracellular space, cytosolOne of 4 routes

• oxalateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

  Catalysed by BCAT2, AADAT, HAO1mitochondrial matrixOne of 3 routes

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

  Catalysed by SLC7A5, AGXT2, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 7 routes

• citric acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC6A14, SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 5 routes

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

  Catalysed by SLC7A5, AGXT2, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 5 routes

• maleateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by SLC6A14, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• tetradecanoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. Myristoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 9 moremitochondrial matrix, cytosolOne of 2 routes

• adipic acidReach from here →
  1. Acetylation of Leucine
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

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

  Catalysed by SLC7A5, AGXT2, AGXTextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• (S)-dihydroorotateReach from here →
  1. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. (S)-dihydroorotate amidohydrolase

  Catalysed by SLC7A13, CADinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• an alcoholReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, CP, PRDX6extracellular space, cytosol

• isocitric acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC6A14, SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• glutarateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  3. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)

  Catalysed by BCAT2, AADAT, SLC22A6mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 9 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. Acetylation of Leucine
  2. ATP citrate lyase (citrate)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by ACLY, GOT2mitochondrial matrix, cytosol

• (2R,3R)-tartrateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by SLC6A14, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes