(S)-methylmalonyl-CoA

Three steps

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  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

• O-acetyl-(R)-carnitineReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by MLYCD, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrixOne of 9 routes

• a 1,2-diacyl-sn-glycero-3-phosphocholineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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

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

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by NAGS, GOT2, GOT1mitochondrial matrix, cytosolOne of 3 routes

• guanidinoacetateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by 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. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2
  2. propanoyl-CoA + H2O = propanoate + CoA + H(+)
  3. propanoate(out) + dehydroepiandrosterone 3-sulfate(in) = propanoate(in) + dehydroepiandrosterone 3-sulfate(out)

  Catalysed by ECHDC1, ACOT8, ACAA2, ACOT9 and 1 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• L-tryptophanReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

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

• L-kynurenineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by KYNUmitochondrial matrix

• beta-alanineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by 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. (S)-methylmalonyl-CoA hydrolase
  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

• leukotriene C4Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. leukotriene C4 hydrolysis

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrix, cytosol

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

  Catalysed by BAAT, GATMmitochondrial matrix, cytosol

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

  Catalysed by BAAT, GADL1, CSADmitochondrial matrix, cytosol

• L-phenylalanineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and phenylalanine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 28 routes

• N(1)-acetylspermineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

  Catalysed by SAT1, SAT2, PAOXmitochondrial matrix, cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by KYNUmitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by NAGS, GCLC, GCLMmitochondrial matrix, cytosolOne of 2 routes

• L-homocysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. L-homocysteine + L-serine = L,L-cystathionine + H2O

  Catalysed by NAGS, CBSmitochondrial matrixOne of 2 routes

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

  Catalysed by NAGS, BHMT2mitochondrial matrix

• 3-oxosphinganineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

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

• L-tyrosineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by GLYAT, GLYATL2, GLYATL1, AADATmitochondrial matrix

• N(1)-acetylspermidineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine + acetyl-CoA = N(1)-acetylspermidine + CoA + H(+)

  Catalysed by SAT1, SAT2mitochondrial matrix, cytosol

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by GLYAT, GLYATL2, GLYATL1, AGXT2mitochondrial matrixOne of 2 routes

• 3-aminopropanalReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. spermidine:oxygen oxidoreductase (spermidine-forming)

  Catalysed by SAT1, SAT2, SMOXmitochondrial matrix, cytosol

• trans-4-hydroxy-L-prolineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 SLC1A4mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(8)-acetylspermidineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  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

• S-sulfanyl-L-cysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by NAGS, CARS2mitochondrial matrix

• a 1,2-diacyl-sn-glycero-3-phosphateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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. (S)-methylmalonyl-CoA hydrolase
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA = itaconyl-CoA + H2O

  Catalysed by SUCLG1, SUCLG2, SUCLA2, AUHmitochondrial matrix

• phosphoenolpyruvateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. ATP citrate lyase (citrate)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by ACLY, PCK1, PCK2mitochondrial matrix, cytosol

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

  Catalysed by NAGS, MSRAmitochondrial matrix

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

  Catalysed by NAGS, MSRB3, MSRB1, MSRB2mitochondrial matrix

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by NAGS, PPCSmitochondrial matrix, cytosolOne of 4 routes

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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

• L-prolineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 SLC1A4mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• estrone 3-sulfateReach from here →
  1. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2
  2. propanoyl-CoA + H2O = propanoate + CoA + H(+)
  3. propanoate(in) + estrone 3-sulfate(out) = propanoate(out) + estrone 3-sulfate(in)

  Catalysed by ECHDC1, ACOT8, ACAA2, ACOT9 and 1 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• sphing-4-enine 1-phosphateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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

• N-benzoylglycineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by NAGS, CDO1mitochondrial matrix, cytosolOne of 2 routes

• (2E)-tetradecenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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