L-leucine

Three steps

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

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

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. transport of leucine and valine (cytosol to extracellular)
  2. L-Valine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

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

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

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. transport of leucine and methionine (cytosol to extracellular)
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by SLC3A2, SLC7A5, AADAT, ADI1extracellular space, cytosol

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A5, MOCOS, RETSATextracellular space, cytosol

• an N-acylsphing-4-enineReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)

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

• N-tetracosanoylsphinganineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

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

• N-hexacosanoylsphinganineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

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

• 3,3',5-triiodo-L-thyronineReach 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 triiodothyronine (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 8 routes

• 1,2,3-tri-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate
  2. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate
  3. 1,2-di-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+) = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O

  Catalysed by PM20D1, DGAT1, LIPA, PNPLA4 and 2 more

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

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosol

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

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

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

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

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

  Catalysed by ALDH6A1, MMUTmitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 4 moremitochondrial matrix, cytosolOne of 4 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. Acetylation of Leucine
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by GLYAT, MCCC1, MCCC2mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

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

• (2E)-glutaconyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by CPT1A, CPT1C, CPT1B, ACOX1mitochondrial matrix, cytosol

• acryloyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Propanoate:CoA ligase (AMP-forming)
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by ACSS3, ACSS2, ACSS1, ACADS and 1 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

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

• benzoyl-CoAReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATextracellular space, cytosol, mitochondrial matrix

• hexadecanoyl-CoAReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATL3extracellular space, cytosolOne of 3 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosolOne of 2 routes

• propanoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. (S)-methylmalonyl-CoA hydrolase
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by ECHDC1mitochondrial matrix

• prostaglandin E1Reach 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 prostaglandin E1 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 2 routes

• prostaglandin I2Reach 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 prostaglandin I2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 2 routes

• prostaglandin F2alphaReach 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 prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 2 routes

• prostaglandin H2Reach 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 prostaglandin H2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 4 routes

• prostaglandin D2Reach 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 prostaglandin D2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by SLC7A5, GSS, GPX5, GPX1 and 7 moreextracellular space, cytosolOne of 3 routes

• S-adenosyl 3-(methylsulfanyl)propylamineReach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by SAT1, SAT2, SRMmitochondrial matrix, cytosol

• 7,8-dihydrofolateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase

  Catalysed by SLC7A5, SHMT1, DHFR, DHFR2extracellular space, cytosolOne of 5 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by SLC7A5, SHMT1, FTCDextracellular space, cytosolOne of 2 routes

• leukotriene A4Reach 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 leukotriene A4 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 3 routes

• (R)-S-lactoylglutathioneReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by SLC7A5, GSS, GLO1, GLOD4extracellular space, cytosolOne of 3 routes

• propane-1,3-diamineReach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by SAT1, SAT2, DHPSmitochondrial matrix, cytosol

• carnosineReach 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 + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC7A5, SLC7A10, CARNS1extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by BCAT2, AADATmitochondrial matrixOne of 3 routes

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

  Catalysed by SLC7A5, TPOextracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC7A5, GFPT2, GFPT1, GNPNAT1extracellular space, cytosolOne of 3 routes

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. Acetylation of Leucine
  2. Palmitate:CoA ligase (AMP-forming)
  3. glycerol-3-phosphate 1-O-acyltransferase (palmitoyl-CoA)

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

• a 1-acylglycerone 3-phosphateReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moremitochondrial matrix, Golgi apparatus

• biotinReach from here →
  1. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)
  2. biocytin + H2O = biotin + L-lysine
  3. Biotinidase (Biotin), Mitochondrial

  Catalysed by SLC7A7, BTDinner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 5 routes

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

  Catalysed by SLC6A14, CPT1A, CPT1C, CPT1Bextracellular space, cytosolOne of 9 routes

• beta-D-fructose 6-phosphateReach from here →
  1. transport of glutamine and leucine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. alpha-D-glucosamine 6-phosphate + H2O = beta-D-fructose 6-phosphate + NH4(+)

  Catalysed by SLC7A5, GFPT2, GFPT1, GNPDA1 and 1 moreextracellular space, cytosol

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

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• S-formylglutathioneReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by SLC7A5, GSS, ADH1C, ADH1A and 4 moreextracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC7A5, AGXT2extracellular space, cytosol, mitochondrial matrix

• succinate semialdehydeReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate

  Catalysed by SLC6A14, SLC6A12, ABATextracellular space, cytosol

• hypotaurineReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by SLC7A9, SLC3A1, CDO1, GADL1 and 1 moreextracellular space, cytosol