3-hydroxyisovaleryl-CoA

Three steps

• leukotriene C4Reach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. leukotriene C4 hydrolysis

  Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrix

• taurocyamineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by CPT1A, CPT1B, BAAT, GATMcytosol

• L-cysteateReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by CPT1A, CPT1B, BAAT, GADL1 and 1 morecytosol

• L-phenylalanineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and phenylalanine (cytosol to extracellular)

  Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrix, extracellular spaceOne of 28 routes

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

  Catalysed by CPT1A, CPT1B, SAT1, SAT2 and 1 morecytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by CPT1A, CPT1B, KYNUcytosol, mitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by CPT1A, CPT1B, NAGS, GCLC and 1 morecytosol, mitochondrial matrixOne of 2 routes

• L-homocysteineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. L-homocysteine + L-serine = L,L-cystathionine + H2O

  Catalysed by CPT1A, CPT1B, NAGS, CBScytosol, mitochondrial matrixOne of 2 routes

• S-methyl-L-methionineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by CPT1A, CPT1B, NAGS, BHMT2cytosol, mitochondrial matrix

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

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 9 morecytosol

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol, peroxisome

• L-tyrosineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrix

• N(1)-acetylspermidineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine + acetyl-CoA = N(1)-acetylspermidine + CoA + H(+)

  Catalysed by CPT1A, CPT1B, SAT1, SAT2cytosol

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrixOne of 2 routes

• 3-aminopropanalReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. spermidine:oxygen oxidoreductase (spermidine-forming)

  Catalysed by CPT1A, CPT1B, SAT1, SAT2 and 1 morecytosol

• trans-4-hydroxy-L-prolineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by CPT1A, CPT1B, SLC1A4cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(8)-acetylspermidineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N8-acetylspermidine to spermidine conversion

  Catalysed by CPT1A, CPT1B, SAT1, SAT2cytosolOne of 3 routes

• N(1),N(12)-diacetylspermineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by CPT1A, CPT1BcytosolOne of 2 routes

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

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 9 morecytosolOne of 2 routes

• S-sulfanyl-L-cysteineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by CPT1A, CPT1B, NAGS, CARS2cytosol, mitochondrial matrix

• a 1,2-diacyl-sn-glycero-3-phosphateReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  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 CPT1A, CPT1B, ASAH1, ACER2 and 15 morecytosol, Golgi apparatus

• (3S)-citramalyl-CoAReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA = itaconyl-CoA + H2O

  Catalysed by CPT1A, CPT1B, SUCLG1, SUCLG2 and 2 morecytosol, mitochondrial matrix

• phosphoenolpyruvateReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. ATP citrate lyase (citrate)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by CPT1A, CPT1B, ACLY, PCK1 and 1 morecytosol

• L-methionine (S)-S-oxideReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by CPT1A, CPT1B, NAGS, MSRAcytosol, mitochondrial matrix

• L-methionine (R)-S-oxideReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by CPT1A, CPT1B, NAGS, MSRB3 and 2 morecytosol, mitochondrial matrix

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by CPT1A, CPT1B, NAGS, PPCScytosol, mitochondrial matrixOne of 4 routes

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  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 CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

• L-prolineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by CPT1A, CPT1B, SLC1A4cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 4 morecytosol, Golgi apparatus

• N-benzoylglycineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by CPT1A, CPT1B, NAGS, CDO1cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol, mitochondrial matrixOne of 5 routes

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

  Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 5 morecytosol, mitochondrial matrixOne of 8 routes

• L-cysteinylglycineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by CPT1A, CPT1B, GGTLC3, GGT1 and 4 morecytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 5 morecytosol, mitochondrial matrixOne of 3 routes

• a cardiolipinReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  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 CPT1A, CPT1B, ASAH1, ACER2 and 3 morecytosol, Golgi apparatus

• (2E)-octenoyl-CoAReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. trans-oct-2-enoyl-CoA reductase

  Catalysed by CPT1A, CPT1B, CROT, MECRcytosol, mitochondrial matrixOne of 5 routes

• 3-oxotetradecanoyl-CoAReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. dodecanoyl-CoA + malonyl-CoA + H(+) = 3-oxotetradecanoyl-CoA + CO2 + CoA

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

• an all-trans-retinyl esterReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. all-trans-retinol + an acyl-CoA = an all-trans-retinyl ester + CoA

  Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 6 morecytosol, Golgi apparatusOne of 4 routes

• 1-O-hexadecyl-sn-glycero-3-phosphocholineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

  Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 3 morecytosol, Golgi apparatus

• a 1,2-diacyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phosphoethanolamine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 6 morecytosol, Golgi apparatus

• a triacyl-sn-glycerolReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + a 1,2-diacyl-sn-glycerol = a triacyl-sn-glycerol + CoA

  Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 8 morecytosol, Golgi apparatus

• a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-acyl-sn-glycero-3-phospho-(1'-sn-glycerol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 5 morecytosol, Golgi apparatus

• 1-octadecanoyl-2-acyl-sn-glycero-3-phospho-1D-myo-inositolReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phospho-D-myo-inositol + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phospho-1D-myo-inositol + CoA

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosol

• 3-oxoeicosasphinganineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + L-serine + H(+) = 3-oxoeicosasphinganine + CO2 + CoA

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 8 morecytosol

• 3-oxoeicosanoyl-CoAReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. very-long-chain 3-oxoacyl-CoA synthase (malonyl-CoA)

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 12 morecytosolOne of 2 routes

• N-docosanoylsphinganineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + sphinganine = N-docosanoylsphinganine + CoA + H(+)

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 9 morecytosol

• N-eicosanoylsphinganineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + sphinganine = N-eicosanoylsphinganine + CoA + H(+)

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 9 morecytosol

• N-(octadecanoyl)-sphinganineReach from here →
  1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. sphinganine + octadecanoyl-CoA = N-(octadecanoyl)-sphinganine + CoA + H(+)

  Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 11 morecytosol