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3-ketoacyl-CoA thiolase, mitochondrial Mevalonate kinase Phosphomevalonate kinase Diphosphomevalonate decarboxylase Isopentenyl- diphosphate Delta-isomerase 1 Farnesyl pyrophosphate synthase Geranylgeranyl pyrophosphate synthase Farnesyl pyrophosphate synthase Geranylgeranyl pyrophosphate synthase Squalene synthase Squalene epoxidase Lanosterol synthase 24- dehydrocholesterol reductase Lanosterol 14-alpha demethylase Delta(14)- sterol reductase 24- dehydrocholesterol reductase Methylsterol monooxygenase 1 Sterol-4- alpha- carboxylate 3- dehydrogenase, decarboxylating HSD17B7 protein Emopamil binding protein (Sterol isomerase) 24- dehydrocholesterol reductase 24- dehydrocholesterol reductase Emopamil binding protein (Sterol isomerase) Emopamil binding protein (Sterol isomerase) Sterol-C5- desaturase (ERG3 delta-5- desaturase homolog, S. cerevisiae)- like 24- dehydrocholesterol reductase 24- dehydrocholesterol reductase 3-hydroxy-3- methylglutaryl- coenzyme A reductase Diacylglycerol O- acyltransferase 1 Gastric triacylglycerol lipase Acetoacetyl-CoA Acetyl-CoA Cholesterol CoA H2O 3-Hydroxy-3-methylglutaryl-CoA CoA Mevalonic acid ATP Mevalonic acid-5P ADP ATP ADP (S)-5-Diphosphomevalonic acid ATP ADP Pi Isopentenyl pyrophosphate CO2 Dimethylallylpyrophosphate Geranyl-PP PPi Farnesyl pyrophosphate PPi NADPH Squalene NADP PPi O2 NADPH (S)-2,3-Epoxysqualene H2O NADP Lanosterol NADPH 24,25-Dihydrolanosterol NADP O2 NADPH 4,4-Dimethylcholesta- 8,14,24-trienol Formic acid NADP H2O NADPH 4,4-Dimethyl-5a-cholesta- 8,24-dien-3-b-ol NADP NADPH 4,4-Dimethyl-5a-cholesta-8- en-3b-ol NADP NADPH O2 4a-Methylzymosterol-4- carboxylic acid NADP H2O NADP 3-Keto-4-methylzymosterol NADPH CO2 NADPH 4a-Methylzymosterol NADP Zymosterol intermediate 2 5a-Cholesta-7,24-dien-3b-ol NADPH 5a-Cholest-8-en-3b-ol NADP NADPH Lathosterol NADP 7-Dehydrodesmosterol 7-Dehydrocholesterol NADPH NADP Desmosterol NADPH O2 NADP H2O NADPH NADP NADPH NADP Vitamin D3 8-Dehydrocholesterol NADPH NADP CoA Fatty acid Cholesterol Ester H2O Fatty acid H2O Magnesium Magnesium Magnesium Magnesium Magnesium Magnesium FAD FAD Heme FAD Iron FAD FAD Iron FAD FAD Butyrate Metabolism Pyruvate Metabolism Valine, Leucine, and Isoleucine Degradation Tryptophan Metabolism Pyruvate Metabolism Fatty Acid Metabolism Steroidogenesis Bile Acid Biosynthesis Endoplasmic Reticulum Peroxisome Peroxisome Intracellular Space Extracellular Space Endoplasmic Reticulum Lysosome hv Spontaneous isomeration
ACAA2 MVK PMVK MVD IDI1 FDPS GGPS1 FDPS GGPS1 FDFT1 SQLE LSS DHCR24 CYP51A1 TM7SF2 DHCR24 MSMO1 NSDHL HSD17B7 EBP DHCR24 DHCR24 EBP EBP SC5D DHCR24 DHCR24 HMGCR DGAT1 LIPF Acetoacetyl-CoA Acetyl-CoA Cholesterol Coenzyme A Water 3-Hydroxy-3- methylglutaryl- CoA Coenzyme A Mevalonic acid Adenosine triphosphate Mevalonic acid-5P Adenosine diphosphate Adenosine triphosphate Adenosine diphosphate (S)-5- Diphosphomevalonic acid Adenosine triphosphate Adenosine diphosphate Phosphate Isopentenyl pyrophosphate Carbon dioxide Dimethylallylpyrophosphate Geranyl-PP Pyrophosphate Farnesyl pyrophosphate Pyrophosphate NADPH Squalene NADP Pyrophosphate Oxygen NADPH (S)-2,3- Epoxysqualene Water NADP Lanosterol NADPH 24,25- Dihydrolanosterol NADP Oxygen NADPH 4,4- Dimethylcholesta- 8,14,24- trienol Formic acid NADP Water NADPH 4,4-Dimethyl- 5a-cholesta- 8,24-dien-3- b-ol NADP NADPH 4,4-Dimethyl- 5a-cholesta- 8-en-3b-ol NADP NADPH Oxygen 4a- Methylzymosterol- 4-carboxylic acid NADP Water NADP 3-Keto-4- methylzymosterol NADPH Carbon dioxide NADPH 4a- Methylzymosterol NADP Zymosterol intermediate 2 5a-Cholesta- 7,24-dien-3b- ol NADPH 5a-Cholest-8- en-3b-ol NADP NADPH Lathosterol NADP 7- Dehydrodesmosterol 7- Dehydrocholesterol NADPH NADP Desmosterol NADPH Oxygen NADP Water NADPH NADP NADPH NADP Vitamin D3 8- Dehydrocholesterol NADPH NADP Coenzyme A Fatty acid Cholesterol Ester Water Fatty acid Water Butyrate Metabolism Pyruvate Metabolism Valine, Leucine, and Isoleucine Degradation Tryptophan Metabolism Pyruvate Metabolism Fatty Acid Metabolism Steroidogenesis Bile Acid Biosynthesis
ACAA2 MVK PMVK MVD IDI1 FDPS GGPS1 FDPS GGPS1 FDFT1 SQLE LSS DHCR24 CYP51A1 TM7SF2 DHCR24 MSMO1 NSDHL HSD17B7 EBP DHCR24 DHCR24 EBP EBP SC5D DHCR24 DHCR24 HMGCR DGAT1 LIPF ActaCoA Ac-CoA Lanol CoA H2O HMG-CoA CoA Mva ATP Mvn-5P ADP ATP ADP S-5DPMA ATP ADP Pi IsopPyr CO2 Ipe Gryl-PP PPi FarPP PPi NADPH Squalen NADP PPi O2 NADPH S23O H2O NADP Lastrol NADPH 2425Di NADP O2 NADPH FF-Mas Formate NADP H2O NADPH 44Dcdol NADP NADPH 44DCBol NADP NADPH O2 4M4CA NADP H2O NADP 3K4Mzol NADPH CO2 NADPH 4aMzmol NADP ZymolI2 5CDBol NADPH 5aChlol NADP NADPH Lathost NADP 7-DHMOL 7DHC NADPH NADP Desmost NADPH O2 NADP H2O NADPH NADP NADPH NADP VitD3 8Dhchol NADPH NADP CoA FA ChoEst H2O FA H2O Mg2+ Mg2+ Mg2+ Mg2+ Mg2+ Mg2+ FAD FAD Heme FAD Iron FAD FAD Iron FAD FAD PW000014 PW000054 PW000051 PW000163 PW000054 PW000023 PW000141 PW000145 Endoplasmic Reticulum Peroxisome Peroxisome Intracellular Space Extracellular Space Endoplasmic Reticulum Lysosome hv Spontaneous isomeration
ACAA2 MVK PMVK MVD IDI1 FDPS GGPS1 FDPS GGPS1 FDFT1 SQLE LSS DHCR24 CYP51A1 TM7SF2 DHCR24 MSMO1 NSDHL HSD17B7 EBP DHCR24 DHCR24 EBP EBP SC5D DHCR24 DHCR24 HMGCR DGAT1 LIPF ActaCoA Ac-CoA Lanol CoA H2O HMG-CoA CoA Mva ATP Mvn-5P ADP ATP ADP S-5DPMA ATP ADP Pi IsopPyr CO2 Ipe Gryl-PP Ppi FarPP Ppi NADPH Squalen NADP Ppi O2 NADPH S23O H2O NADP Lastrol NADPH 2425Di NADP O2 NADPH FF-Mas Formate NADP H2O NADPH 44Dcdol NADP NADPH 44DCBol NADP NADPH O2 4M4CA NADP H2O NADP 3K4Mzol NADPH CO2 NADPH 4aMzmol NADP ZymolI2 5CDBol NADPH 5aChlol NADP NADPH Lathost NADP 7-DHMOL 7DHC NADPH NADP Desmost NADPH O2 NADP H2O NADPH NADP NADPH NADP VitD3 8Dhchol NADPH NADP CoA FA ChoEst H2O FA H2O PW000014 PW000054 PW000051 PW000163 PW000054 PW000023 PW000141 PW000145