isobutyryl-CoA

Three steps

• sphingosine 1-phosphateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 5 moremitochondrial matrix, Golgi apparatus, cytosolOne of 2 routes

• glycolithocholic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by GLYAT, GLYATL2, GLYATL1, BAATmitochondrial matrix, cytosolOne of 2 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, GCATmitochondrial matrix

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Production of Glutaryl Carnitine
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 2 moremitochondrial matrix, cytosol

• phosphoenolpyruvic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. ATP citrate lyase (citrate)
  3. GTP:oxaloacetate carboxy-lyase (adding GTP

  Catalysed by GLYAT, GLYATL2, ACLY, PCK1mitochondrial matrix, cytosolOne of 2 routes

• 13-cis-retinolReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 13-cis-retinol

  Catalysed by GLYAT, GLYATL2, DGAT1, LRAT and 4 moremitochondrial matrix, cytosol

• spermineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

  Catalysed by GLYAT, GLYATL2, SAT1, SAT2 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 3 moremitochondrial matrix, cytosol, endoplasmic reticulum

• L-ornithineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by GLYAT, GLYATL2, BAAT, GATMmitochondrial matrix, cytosolOne of 15 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  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 GLYAT, GLYATL2, ASAH1, ACER2 and 3 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• a 1,3-diacylglycerolReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 4 moremitochondrial matrix, Golgi apparatus

• a 1,2-diacylglycerolReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 7 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• gamma-Glu-AlaReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by GLYAT, GLYATL2, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GLDC and 1 moremitochondrial matrix

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

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 8 moremitochondrial matrix, Golgi apparatus

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

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 9 moremitochondrial matrix, cytosol

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

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 9 moremitochondrial matrix, cytosol

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  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 GLYAT, GLYATL2, ASAH1, ACER2 and 5 moremitochondrial matrix, Golgi apparatus

• succinyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  3. (R)-methylmalonyl-CoA = succinyl-CoA

  Catalysed by GLYAT, GLYATL2, ALDH6A1, MMUTmitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by GLYAT, GLYATL2, ACSM4, ACSM1 and 6 moremitochondrial matrix, cytosolOne of 4 routes

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

  Catalysed by GLYAT, GLYATL2, MCCC1, MCCC2mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosol

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

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 2 moremitochondrial matrix, cytosol

• acryloyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Propanoate:CoA ligase (AMP-forming)
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by GLYAT, GLYATL2, ACSS3, ACSS2 and 3 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  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 GLYAT, GLYATL2, SLC27A2, ACSL4 and 7 moremitochondrial matrix, cytosol

• hexadecanoyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Carnitine O-Palmitoyltransferase
  3. (2E)-hexadecenoyl-CoA + NADPH + H(+) = hexadecanoyl-CoA + NADP(+)

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 2 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  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 GLYAT, GLYATL2, SLC27A2, ACSL4 and 7 moremitochondrial matrix, cytosol

• propanoyl-CoAReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. (S)-methylmalonyl-CoA hydrolase
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by GLYAT, GLYATL2, ECHDC1mitochondrial matrix

• D-alanineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by GLYAT, GLYATL2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by GLYAT, GLYATL2, GLYATL1, PIPOXmitochondrial matrixOne of 2 routes

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

  Catalysed by GLYAT, GLYATL2, SAT1, SAT2 and 1 moremitochondrial matrix, cytosol

• 7,8-dihydrofolateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by GLYAT, GLYATL2, BAAT, MTO1mitochondrial matrix, cytosol

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by GLYAT, GLYATL2, GLYATL1, SHMT1 and 1 moremitochondrial matrix

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

  Catalysed by GLYAT, GLYATL2, SAT1, SAT2 and 1 moremitochondrial matrix, cytosol

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

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 10 moremitochondrial matrix, cytosol

• a 1-acylglycerone 3-phosphateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 3 moremitochondrial matrix, Golgi apparatus

• O-acetyl-(R)-carnitineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Carnitine O-Palmitoyltransferase
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 9 routes

• a 1,2-diacyl-sn-glycero-3-phosphocholineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  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 GLYAT, GLYATL2, ASAH1, ACER2 and 3 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• an N-acylglycineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 3 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by GLYAT, GLYATL2, NAGS, GOT2 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• guanidinoacetateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. L-Arginine:glycine amidinotransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, GATMmitochondrial matrix, cytosolOne of 2 routes

• D-threonineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by GLYAT, GLYATL2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  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 GLYAT, GLYATL2, ASAH1, ACER2 and 4 moremitochondrial matrix, Golgi apparatus

• L-tryptophanReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and tryptophan (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 20 routes

• glutathioneReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GSSmitochondrial matrix

• L-threonineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by GLYAT, GLYATL2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-kynurenineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by GLYAT, GLYATL2, KYNUmitochondrial matrix

• beta-alanineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by GLYAT, GLYATL2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 3 routes

• a 1-acyl-sn-glycero-3-phosphateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sn-glycerol 3-phosphate + an acyl-CoA = a 1-acyl-sn-glycero-3-phosphate + CoA

  Catalysed by GLYAT, GLYATL2, ASAH1, ACER2 and 6 moremitochondrial matrix, Golgi apparatus