isobutyryl-CoA

Three steps

• N-acetyl-L-cysteineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by GLYAT, GLYATL2, NAGSmitochondrial matrix

• K(+)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by GLYAT, GLYATL2, NAGS, SLC1A1mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• Cys-tRNA(Cys)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by GLYAT, GLYATL2, NAGS, CARS1mitochondrial matrix, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GARS1mitochondrial matrix, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-asparagine)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by GLYAT, GLYATL2, NAGS, NARS1mitochondrial matrix, cytosol

• chlorine(.)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Carnitine O-Palmitoyltransferase
  3. Carnitine Transport by Atb0

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 2 moremitochondrial matrix, cytosol, extracellular spaceOne of 15 routes

• phosphocholineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by GLYAT, GLYATL2, CHAT, PHOSPHO1mitochondrial matrix, nucleusOne of 2 routes

• glycocholateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. propanoyl-CoA C-acyltransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by GLYAT, GLYATL2, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisomeOne of 5 routes

• cholateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. propanoyl-CoA C-acyltransferase
  3. choloyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisome, cytosolOne of 2 routes

• hydrogen sulfideReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by GLYAT, GLYATL2, NAGS, CTHmitochondrial matrix, cytosolOne of 2 routes

• L-aspartateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by GLYAT, GLYATL2, AGXT2mitochondrial matrixOne of 12 routes

• (9Z,12Z)-octadecadienoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

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

• 3-acetamidopropanalReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by GLYAT, GLYATL2, PAOX, SMOXmitochondrial matrix, cytosolOne of 4 routes

• propionic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Propanoate:CoA ligase (AMP-forming)
  3. Propanoyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, ACSS3, ACSS2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• citric acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. ATP citrate lyase (citrate)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

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

• butyric acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. Butyryl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, ACSM4, ACSM1 and 5 moremitochondrial matrix, cytosol

• tetradecanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. Myristoyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 11 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes

• adipic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by GLYAT, GLYATL2, ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• glutarateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + H2O = glutarate + CoA + H(+)

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 3 moremitochondrial matrix, cytosolOne of 5 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. ATP citrate lyase (citrate)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by GLYAT, GLYATL2, ACLY, GOT2mitochondrial matrix, cytosol

• docosan-1-olReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 7 moremitochondrial matrix, cytosol

• hexacosanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. Hexacosanoyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosolOne of 2 routes

• tetracosanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. Tetracosenoyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• an N-acylsphinganineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphingosine N-acyltransferase (sphinganine)

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

• octadecan-1-olReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 7 moremitochondrial matrix, cytosol

• eicosanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. Eicosanoyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 9 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 8 routes

• (15Z)-tetracosenoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)
  3. (15Z)-tetracosenoyl-CoA hydrolase

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

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 4 routes

• (11Z)-eicosenoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
  3. (11Z)-eicosenoyl-CoA hydrolase

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

• putrescineReach 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, cytosolOne of 3 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by GLYAT, GLYATL2, NAGS, IL4I1mitochondrial matrix, cytosol

• di-mu-sulfido-diiron(2+)Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. De novo synthesis of 2Fe2S

  Catalysed by GLYAT, GLYATL2, NAGS, NDUFAB1 and 6 moremitochondrial matrix

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

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

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

  Catalysed by GLYAT, GLYATL2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 6 routes

• an acylcholineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by GLYAT, GLYATL2, CHAT, BCHEmitochondrial matrix, nucleus

• fatty acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. long-chain-fatty-acid-CoA ligase (elaidate)
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GLYAT, GLYATL2, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• 5-aminolevulinateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by GLYAT, GLYATL2, GLYATL1, ALAS1 and 1 moremitochondrial matrix

• chenodeoxycholateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. chenodeoxycholoyl-CoA hydrolase

  Catalysed by GLYAT, GLYATL2, ACAA1, SCP2 and 4 moremitochondrial matrix, peroxisomeOne of 3 routes

• glycochenodeoxycholateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, BAATmitochondrial matrix

• taurocholateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. propanoyl-CoA C-acyltransferase
  3. Choloyl-CoA:glycine N-choloyltransferase

  Catalysed by GLYAT, GLYATL2, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisomeOne of 5 routes

• taurodeoxycholateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by GLYAT, GLYATL2, SLC27A2, SLC27A5 and 1 moremitochondrial matrix, cytosolOne of 4 routes

• glycochenodeoxycholic acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by GLYAT, GLYATL2, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisomeOne of 5 routes

• cholesteryl hexadecanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Acyl-CoA:cholesterol O-acyltransferase

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

• glyoxylateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by GLYAT, GLYATL2, GLYATL1, AADATmitochondrial matrixOne of 11 routes

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

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

• CHEBI:36882Reach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)

  Catalysed by GLYAT, GLYATL2, SUCLG1, SUCLG2 and 2 moremitochondrial matrix

• 3-hydroxyisovaleric acidReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  3. Formation of 3-Hydroxy-Isovalerate

  Catalysed by GLYAT, GLYATL2, CPT1A, CPT1Bmitochondrial matrix, cytosol

• 3,7,11,15-tetramethylhexadecanoateReach from here →
  1. glycine N-acyltransferase (isobutyrylglycine)
  2. phytanate-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by GLYAT, GLYATL2, ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 3 routes