L-isoleucine

Three steps

• O-decanoyl-(R)-carnitineReach from here →
  1. Acetylation of Isoleucine
  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

• 3-disulfanyl-L-alanineReach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by SLC7A5, SLC7A9, SLC3A1, CTHextracellular space, cytosol

• CHEBI:28862Reach from here →
  1. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)
  2. glycyl-tRNA(gly) phosphorylation
  3. 2'-deoxyguanosine 5'-diphosphate:oxidized-thioredoxin 2'-oxidoreductase

  Catalysed by IARS1, TXN, RRM1, RRM2 and 2 morecytosol

• a dicarboxylateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation
  3. N-benzoylglycine(out) + a dicarboxylate(in) = N-benzoylglycine(in) + a dicarboxylate(out)

  Catalysed by SLC7A5, SLC22A6, SLC22A8extracellular space, cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-ascorbic acidReach from here →
  1. transport of glycine and isoleucine (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 isoleucine (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 SLC7A5, SLC1A1, SLC24A1, SLC24A5 and 3 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. transport of isoleucine and leucine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

  Catalysed by SLC7A5, BCAT2, BCKDHA, BCKDHBextracellular space, cytosol, mitochondrial matrix

• Asp-tRNA(Asp)Reach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by SLC7A5, AGXT2, DARS1extracellular space, cytosol

• hypoioditeReach from here →
  1. transport of isoleucine 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 isoleucine (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 SLC7A5, CTH, SUMF1extracellular space, cytosol

• hydrogenselenideReach from here →
  1. transport of alanine and isoleucine (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 isoleucine (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 isoleucine (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. transport of glycine and isoleucine (cytosol to extracellular)
  2. glycine:oxygen oxidoreductase (deaminating)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by SLC7A5, DAO, GRHPRextracellular space, cytosol, peroxisomeOne of 3 routes

• D-aspartateReach from here →
  1. transport of cysteine and isoleucine (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 SLC7A5, SLC1A1, SLC1A7, SLC1A3 and 2 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• acetateReach from here →
  1. transport of alanine and isoleucine (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 9 routes

• (9Z,12Z)-octadecadienoateReach from here →
  1. Acetylation of Isoleucine
  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 isoleucine (cytosol to extracellular)
  2. Transport by Y+Lat-2 into Extracellular Space
  3. N1-acetylspermidine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by SLC7A6, SLC3A2, PAOX, SMOXextracellular space, cytosolOne of 4 routes

• oxalateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

  Catalysed by SLC7A5, AADAT, HAO1extracellular space, cytosolOne of 3 routes

• anthranilic acidReach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. L-Kynurenine hydrolase

  Catalysed by SLC7A5, KYNUextracellular space, cytosol

• propionic acidReach from here →
  1. transport of alanine and isoleucine (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 isoleucine (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 isoleucine (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 isoleucine (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 Isoleucine
  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

• (9Z)-octadecenoateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. (9Z)-octadecenoate + glycine = N-(9Z-octadecenoyl)glycine + H2O

  Catalysed by SLC7A5, GLYATL3, GLYATL2, FAAH and 1 moreextracellular space, cytosolOne of 6 routes

• adipic acidReach from here →
  1. Acetylation of Isoleucine
  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 isoleucine (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

• an alcoholReach from here →
  1. transport of glycine and isoleucine (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 isoleucine (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. transport of glycine and isoleucine (cytosol to extracellular)
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycocholate(out) + glutarate(in) = glycocholate(in) + glutarate(out)

  Catalysed by SLC7A5, BAAT, SLC22A24extracellular space, cytosol, peroxisome, inner side of the membrane, outer side of the membraneOne of 7 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. Acetylation of Isoleucine
  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 isoleucine (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

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

• (3R)-citramalateReach from here →
  1. transport of chloride and isoleucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

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

• (3S)-citramalateReach from here →
  1. transport of chloride and isoleucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

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

• docosan-1-olReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

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

• hexacosanoateReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. Hexacosanoyl-CoA hydrolase

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

• tetracosanoateReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. Tetracosenoyl-CoA hydrolase

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

• an N-acylsphinganineReach from here →
  1. Acetylation of Isoleucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphingosine N-acyltransferase (sphinganine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moremitochondrial matrix, Golgi apparatus, cytosol

• octadecan-1-olReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

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

• eicosanoateReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. Eicosanoyl-CoA hydrolase

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

• (9Z)-hexadecenoateReach from here →
  1. transport of chloride and isoleucine (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A14extracellular space, cytosol, endoplasmic reticulumOne of 8 routes

• (15Z)-tetracosenoateReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)
  3. (15Z)-tetracosenoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by SLC7A5, GSSextracellular space, cytosolOne of 8 routes

• (11Z)-eicosenoateReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
  3. (11Z)-eicosenoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosol

• cis-aconitic acidReach from here →
  1. transport of chloride and isoleucine (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

• N-carbamoyl-L-aspartateReach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

  Catalysed by SLC7A5, AGXT2, CADextracellular space, cytosolOne of 2 routes

• 3-oxopropanoateReach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by SLC7A5, AGXT2extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• thiosulfateReach from here →
  1. transport of cysteine and isoleucine (cytosol to extracellular)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate:sulfide sulfurtransferase

  Catalysed by SLC7A5, GOT2, GOT1, MPST and 1 moreextracellular space, cytosolOne of 6 routes