L-leucine

Three steps

• 3-fumarylpyruvateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by SLC7A5, AGXT2, FAHD1extracellular space, cytosol, mitochondrial matrix

• pantetheine 4'-phosphateReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4'-Phosphopantothenoyl]-L-cysteine carboxy-lyase

  Catalysed by SLC7A9, SLC3A1, PPCS, PPCDCextracellular space, cytosolOne of 4 routes

• psychosineReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2mitochondrial matrix, Golgi apparatus, cytosol

• trans-1,2-dithiane-4,5-diolReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by SLC7A5, SCLYextracellular space, cytosol

• creatineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by SLC7A5, GATM, GAMTextracellular space, cytosolOne of 2 routes

• 1-acyl-sn-glycerol 3-phosphateReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moremitochondrial matrix, Golgi apparatus

• bilirubin IXalphaReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of bilirubin and GSH (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 2 routes

• a cholesterol esterReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + cholesterol = a cholesterol ester + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moremitochondrial matrix, Golgi apparatus

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

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

• 2-aminoacrylic acidReach from here →
  1. transport of leucine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. thyroid peroxidase (3,5-diiodo-L-tyrosine)

  Catalysed by SLC7A5, TPOextracellular space, cytosol

• taurolithocholateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and taurolithocholate (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 4 routes

• 3-hydroxypyruvateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

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

• CHEBI:17208Reach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by SLC7A5, GSSextracellular space, cytosol

• (S)-4-hydroxymandelateReach from here →
  1. transport of leucine and tyrosine (cytosol to extracellular)
  2. L-tyrosine:2-oxoglutarate aminotransferase
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by SLC7A5, GOT2, GOT1, TAT and 1 moreextracellular space, cytosol

• homocysteineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Formation of Homocystine

  Catalysed by SLC7A5, GSSextracellular space, cytosol

• S-(4,8-dimethylnonanoyl)-4'-phosphopantetheineReach from here →
  1. Acetylation of Leucine
  2. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal
  3. 4,8-dimethylnonanoyl-CoA + H2O = S-(4,8-dimethylnonanoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by CRAT, CROT, NUDT19mitochondrial matrix, peroxisome

• S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (linolenate)
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + H2O = S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• S-(9Z-hexadecenoyl)-4'-phosphopantetheineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (palmitolate)
  3. (9Z)-hexadecenoyl-CoA + H2O = S-(9Z-hexadecenoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• itaconateReach from here →
  1. Acetylation of Leucine
  2. Itaconate:CoA ligase (GDP-forming)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 4 routes

• N-octadecanoyl-heptadecasphing-4-enineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. heptadecasphing-4-enine + octadecanoyl-CoA = N-octadecanoyl-heptadecasphing-4-enine + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• dehydroascorbic acidReach from here →
  1. solute carrier (L-dopa)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. dopamine,ascorbate:oxygen oxidoreductase (beta-hydroxylating)

  Catalysed by SLC3A2, SLC7A5, DDC, DBHextracellular space, cytosol

• aceneuramateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by SLC7A5, AGXT2, NPLextracellular space, cytosol, mitochondrial matrix

• 5'-deoxyadenosineReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by SLC7A5, AGXT2, TYW1B, TYW1extracellular space, cytosol, mitochondrial matrixOne of 5 routes

• all-trans-retinolReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, RETSATextracellular space, cytosolOne of 3 routes

• linoleic 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 3 routes

• sulfiteReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by SLC7A5, GSS, TSTD1extracellular space, cytosolOne of 6 routes

• D-aspartic acidReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by SLC6A14, SLC12A2, SLC12A1, SLC1A7 and 4 moreextracellular space, cytosol

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moremitochondrial matrix, Golgi apparatus, cytosol

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by SLC6A14, CPT2, CPT1Aextracellular space, cytosol, peroxisomeOne of 14 routes

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. Acetylation of Leucine
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. Lecithin Retinol Acyltransferase (11-Cis)

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

• S-methyl-5'-thioadenosineReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by SLC3A2, SLC7A6, SLC7A7, SRMextracellular space, cytosolOne of 4 routes

• diiodineReach from here →
  1. transport of leucine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by SLC7A5, TPOextracellular space, cytosolOne of 2 routes

• all-trans-retinyl hexadecanoateReach from here →
  1. Acetylation of Leucine
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. all-trans-retinyl hexadecanoate + H2O = 11-cis-retinol + hexadecanoate + H(+)

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 2 moremitochondrial matrix, cytosol

• nitrateReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

  Catalysed by SLC3A2, SLC7A6, SLC7A7, NOS3 and 3 moreextracellular space, cytosol

• 3,3',5'-triiodo-L-thyronine sulfateReach 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. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by SLC7A5, MOCOS, DIO1extracellular space, cytosol

• 3,3'-diiodo-L-thyronineReach 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. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach 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. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by SLC7A5, MOCOS, DIO1extracellular space, cytosolOne of 4 routes

• CHEBI:17724Reach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by SLC7A5, GNMT, PDPR, DMGDHextracellular space, cytosolOne of 2 routes

• CHEBI:17769Reach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC3A2, SLC7A6, SLC7A7, AOC2 and 2 moreextracellular space, cytosolOne of 3 routes

• urateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by SLC7A5, GSS, SLC22A13extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• a 1,2-diacyl-sn-glycerolReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacyl-sn-glycerol + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moremitochondrial matrix, Golgi apparatus

• methylarsonous 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. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, GSTO1, GSTO2extracellular space, cytosol

• a triacylglycerolReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moremitochondrial matrix, Golgi apparatus, cytosol

• 3-oxopropanoic acidReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A5, SLC7A10, ABATextracellular space, cytosol, inner side of the membrane, outer side of the membrane, mitochondrial matrix

• butanoateReach from here →
  1. Acetylation of Leucine
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + H2O = butanoate + CoA + H(+)

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 8 moremitochondrial matrix, cytosol

• fumaric acidReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by SLC3A2, SLC7A6, SLC7A7, ASLextracellular space, cytosolOne of 8 routes

• thiocyanateReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by SLC7A9, SLC3A1, GOT2, GOT1 and 2 moreextracellular space, cytosol

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

  Catalysed by SLC7A5, AGXT2, PKM, PKLRextracellular space, cytosol, mitochondrial matrix

• indole-3-acetaldehydeReach from here →
  1. transport of leucine and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase
  3. Tryptamine:oxygen oxidoreductase(deaminating)

  Catalysed by SLC7A5, DDC, AOC1, MAOA and 1 moreextracellular space, cytosolOne of 2 routes