L-leucine

Three steps

• N-sulfo-D-glucosamineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by SLC6A14, SLC26A6, SGSHextracellular space, cytosol

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

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

• dehydroepiandrosterone 3-sulfateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by SLC6A14, SLC26A6, STSextracellular space, cytosol

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

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

• dimethylamineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by SLC7A5, AGXT2, DDAH1extracellular space, cytosolOne of 2 routes

• IMPReach from here →
  1. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  2. IMP:L-aspartate ligase (GDP-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by SLC7A13, ADSS2, ADSS1inner side of the membrane, outer side of the membrane, cytosol

• L-cysteateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by SLC6A14, SLC6A6, GADL1, CSADextracellular space, cytosol

• N(1)-acetylspermineReach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

  Catalysed by SAT1, SAT2, PAOXmitochondrial matrix, cytosol

• porphobilinogenReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by SLC7A5, ALAS1, ALAS2, CPNE7 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• phosphoethanolamineReach from here →
  1. transport of leucine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. phosphoethanolamine + H2O = acetaldehyde + NH4(+) + phosphate

  Catalysed by SLC7A5, ETNPPLextracellular space, cytosol

• adenosine 5'-phosphosulfateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. ATP:sulfate adenylyltransferase

  Catalysed by SLC6A14, SLC26A6, PAPSS1, PAPSS2extracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by SLC7A5, PIPOX, DMGDHextracellular space, cytosolOne of 3 routes

• N-acetylputrescineReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. acetyl-CoA:putrescine N-acetyltransferase

  Catalysed by SLC3A2, SLC7A6, SLC7A7, SAT1 and 1 moreextracellular space, cytosolOne of 2 routes

• 4-aminobutanalReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. putrescine + O2 + H2O = 4-aminobutanal + H2O2 + NH4(+)

  Catalysed by SLC3A2, SLC7A6, SLC7A7, AOC1extracellular space, cytosol

• L-glutamyl 5-phosphateReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate
  3. L-glutamate 5-semialdehyde + phosphate + NADP(+) = L-glutamyl 5-phosphate + NADPH + H(+)

  Catalysed by SLC7A6, OAT, ALDH18A1extracellular space, cytosol

• 3-oxosphinganineReach from here →
  1. Acetylation of Leucine
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

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

• taurolithocholate 3-sulfateReach 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 sulfotaurolithocholate (cytosol to extracellular)

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

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. Acetylation of Leucine
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

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

• (S)-2-amino-6-oxohexanoateReach from here →
  1. transport of leucine and lysine (cytosol to extracellular)
  2. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)
  3. L-saccharopine + NAD(+) + H2O = (S)-2-amino-6-oxohexanoate + L-glutamate + NADH + H(+)

  Catalysed by SLC7A6, AASSextracellular space, cytosol, mitochondrial matrix

• N(1)-acetylspermidineReach from here →
  1. transport of arginine and leucine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2

  Catalysed by SLC3A2, SLC7A6, SLC7A7, PAOXextracellular space, cytosolOne of 2 routes

• 3-aminopropanalReach from here →
  1. transport of leucine and lysine (cytosol to extracellular)
  2. deoxyhypusine synthase (1,3-diaminopropane)
  3. primary-amine oxidase (1,3-diaminopropane)

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

• 5-oxo-L-prolineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. glutathione = L-cysteinylglycine + 5-oxo-L-proline

  Catalysed by SLC7A5, GSS, CHAC2, CHAC1extracellular space, cytosolOne of 2 routes

• L-thyroxineReach 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 thyroxine (cytosol to extracellular)

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

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by SLC7A5, SHMT1, ATICextracellular space, cytosolOne of 3 routes

• N(8)-acetylspermidineReach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N8-acetylspermidine to spermidine conversion

  Catalysed by SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

• N(1),N(12)-diacetylspermineReach from here →
  1. Acetylation of Leucine
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  No catalyst recordedmitochondrial matrix, cytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosolOne of 2 routes

• N(tau)-methylhistamineReach from here →
  1. transport of histidine and leucine (cytosol to extracellular)
  2. L-histidine carboxy-lyase (histamine-forming)
  3. S-Adenosyl-L-methionine:histamine N-tele-methyltransferase

  Catalysed by SLC7A6, HDC, HNMTextracellular space, cytosolOne of 3 routes

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

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 13 moremitochondrial matrix, Golgi apparatus

• (3S)-citramalyl-CoAReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

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

• phosphoenolpyruvateReach from here →
  1. Acetylation of Leucine
  2. ATP citrate lyase (citrate)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by ACLY, PCK1, PCK2mitochondrial matrix, cytosol

• S-sulfanylglutathioneReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by SLC7A5, GSS, SQORextracellular space, cytosol, mitochondrial matrixOne of 8 routes

• methylamineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. N(omega)-methyl-L-arginine + glyoxylate = 5-(3-methylguanidino)-2-oxopentanoate + glycine
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

  Catalysed by SLC7A5, AGXT2, DDAH1extracellular space, cytosol

• monodehydro-L-ascorbate radicalReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. N-(9Z-octadecenoyl)glycine + 2 L-ascorbate + O2 = N-(9Z-octadecenoyl)-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by SLC7A5, GLYATL3, GLYATL2, PAMextracellular space, cytosolOne of 6 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

  Catalysed by SLC7A5, AADAT, PAHextracellular space, cytosolOne of 4 routes

• pyridoxal 5'-phosphateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by SLC7A5, SCLY, PNPOextracellular space, cytosol

• an N-acyl-L-amino acidReach from here →
  1. Transport of L-Kynurenine
  2. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)
  3. an N-acyl-L-amino acid + H2O = an L-alpha-amino acid + a carboxylate

  Catalysed by SLC43A1, SLC7A11, ACY1, PM20D1extracellular space, cytosol, inner side of the membrane, outer side of the membrane

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + a 1-acyl-sn-glycero-3-phosphocholine = 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

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

• glycolithocholate 3-sulfateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. bile acid-CoA:amino acid N-acyltransferase (glycine)
  3. sulfotransferase (glycolithocholate)

  Catalysed by SLC7A5, BAAT, SULT2A1extracellular space, cytosolOne of 3 routes

• a 2,3-dehydroacyl-CoAReach 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. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, ACAD10extracellular space, cytosol

• estrone 3-sulfateReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by SLC6A14, SLC26A6, STSextracellular space, cytosol

• sphing-4-enine 1-phosphateReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

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

• prostaglandin E2Reach 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 E2 (cytosol to extracellular)

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

• (2E)-tetradecenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. trans-tetradec-2-enoyl-CoA reductase

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

• (2E)-decenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. trans-dec-2-enoyl-CoA reductase

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 3 moremitochondrial matrix, cytosolOne of 8 routes

• Fe(II)-heme aReach 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. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, COX15extracellular space, cytosol

• (R)-4'-phosphopantetheineReach 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 + H(+) = (R)-4'-phosphopantetheine + CO2

  Catalysed by SLC7A9, SLC3A1, PPCS, PPCDCextracellular space, cytosol

• (2E)-hexenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. medium-chain acyl-CoA ligase (hexanoic acid)
  3. trans-hex-2-enoyl-CoA reductase

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

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by BCAT2, AADAT, HOGA1mitochondrial matrixOne of 2 routes

• a cardiolipinReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + an acyl-CoA = a cardiolipin + CoA

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