Pathways

The biosynthesis of Cholesterol starts with acetyl-CoA reacts with acetyl-CoA c-acetyltransferase resulting in the release of CoA acetoacetyl-CoA, The latter compound then reacts with an acetyl-coa through a hydroxymethylglutaryl-CoA synthase resulting in the release of 3-hydroxy-3-methylglutaryl-CoA. The latter compound in turn reacts with a NADPH through a 3-hydroxy-3-methylglutaryl-coenzyme A reductase resulting in the release of a NADP, Coenzyme A and Mevalonic acid. The latter is then phosphorylated by ATP through a mevalonate kinase resulting in the release of ADP and Mevalonic acid-5P which is then phosphorylated by ATP through a phosphomevalonate kinase resulting in the release of ADP and (S)-5-diphosphomevalonic acid. The latter compound in turn reacts with ATP through a diphosphomevalonic decarboxylase resulting in the release of phosphate, ADP, carbon dioxide and Isopentenyl pyrophosphate. The latter compound in turn reacts with isopentenyl diphosphate delta isomerase resulting in the release of dimethylallylpyrophosphate. The latter compound then reacts with isopentenyl pyrophosphate through a farnesyl pyrophosphate synthase resulting in the release of Geranyl-PP. The latter then reacts with an isopentenyl pyrophosphate through farnesyl pyrophosphate synthase resulting in the release of pyrophospate and farnesyl pyrophosphate. Farnesyl pyrophosphate then reacts with NADPH through a squalene synthase in order to produce squalene while also releasing two phosphates and NADP. Squalene then reacts with oxygen and NADPH through a squalene monooxygenase resulting in the release of water, NADP and (S)-2,3-epoxysqualene. The latter in turn reacts with lanosterol synthase resulting in the release of lanosterin. Lanosterin then reacts with oxygen and NADPH through a lanosterol 14-alpha demethylase resulting in the release of formic acid, water, NADP and 4,4-dimethylcholesta-8,14,24-trienol. The latter compound in turn is reduced by an NADPH through a Delta (14)-sterol reductase resulting in the release of NADP and 4,4-dimethyl-5a-cholesta-8,24-dien-3-b-ol. The latter reacts with hydrogen ion,oxygen and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-hydroxymethyl-4B-methyl-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with a hydrogen ion, water, and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-formyl-4b-methyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen, NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4B-methyl-4a-carboxy-cholesta-8,24-dien-3B-ol. The latter reacts with an NADP through c-3 sterol dehydrogenase resulting in the release of NADPH, carbon dioxide and 3-keto-4-methylzymosterol. The latter is reduced by NADPH through a 3-keto sterol reductase resulting in the release of NADP and 4a-methylzymosterol. The latter then reacts with hydrogen, oxygen and nadph through methylsterol monooxygenase resulting in the release of water, NADP and 4a-hydroxymethyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with water, hydrogen and NADPH through a methylsterol monooxygenase resulting in the release of water, NADP and 4a-formyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen and NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4a-carboxy-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with NADP through a C-3 sterol dehydrogenase resulting in the release of carbon dioxide, NADPH and 5a-cholesta-8,24-dien-3-one. The latter reacts with hydrogen ion and NADPH through a 3-keto sterol reductase resulting in the release of NADP and zymosterol. Zymosterol can either be used to create ergosterol starts with zymosterol reacting with S-adenosylmethionine through a sterol 24-c-methyltransferase resulting in the release of S-adenosylhomocysteine, hydrogen ion and fecosterol. Fecosterol reacts with C-8 sterol isomerase resulting in the release of episterol. Episterol reacts with oxygen, hydrogen ion and ferrocytochrome c through a C-5 sterol desaturase resulting in the release of ferricytochrome c, water and 5,7,24(28)-ergostatrienol. The latter reacts with hydrogen ion, oxygen, NADPH and c-22 sterol desaturase resulting in the release of water, NADP AND ERGOSTA-5,7,22,24(28)-tetraen-3-B-ol. The latter compound reacts with hydrogen ion and NADPH through a C-24 sterol reductase resulting in the release of NADP and ergosterol. Zymosterol reacts with C-8 sterol isomerase resulting in the release of 5a-cholesta-7,24-dien-3b-ol. The latter compound reacts with C-5 sterol desaturase resulting in the release of 7-dehydrodesmosterol. The latter is then converted spontaneously through desmosterol. Desmosterol is then spontaneously turned into cholesterol which can in turn react with Dodecanoic acid spontaneously resulting in the release of Coenzyme A and CE(12:0).

The biosynthesis of Cholesterol starts with acetyl-CoA reacts with acetyl-CoA c-acetyltransferase resulting in the release of CoA acetoacetyl-CoA, The latter compound then reacts with an acetyl-coa through a hydroxymethylglutaryl-CoA synthase resulting in the release of 3-hydroxy-3-methylglutaryl-CoA. The latter compound in turn reacts with a NADPH through a 3-hydroxy-3-methylglutaryl-coenzyme A reductase resulting in the release of a NADP, Coenzyme A and Mevalonic acid. The latter is then phosphorylated by ATP through a mevalonate kinase resulting in the release of ADP and Mevalonic acid-5P which is then phosphorylated by ATP through a phosphomevalonate kinase resulting in the release of ADP and (S)-5-diphosphomevalonic acid. The latter compound in turn reacts with ATP through a diphosphomevalonic decarboxylase resulting in the release of phosphate, ADP, carbon dioxide and Isopentenyl pyrophosphate. The latter compound in turn reacts with isopentenyl diphosphate delta isomerase resulting in the release of dimethylallylpyrophosphate. The latter compound then reacts with isopentenyl pyrophosphate through a farnesyl pyrophosphate synthase resulting in the release of Geranyl-PP. The latter then reacts with an isopentenyl pyrophosphate through farnesyl pyrophosphate synthase resulting in the release of pyrophospate and farnesyl pyrophosphate. Farnesyl pyrophosphate then reacts with NADPH through a squalene synthase in order to produce squalene while also releasing two phosphates and NADP. Squalene then reacts with oxygen and NADPH through a squalene monooxygenase resulting in the release of water, NADP and (S)-2,3-epoxysqualene. The latter in turn reacts with lanosterol synthase resulting in the release of lanosterin. Lanosterin then reacts with oxygen and NADPH through a lanosterol 14-alpha demethylase resulting in the release of formic acid, water, NADP and 4,4-dimethylcholesta-8,14,24-trienol. The latter compound in turn is reduced by an NADPH through a Delta (14)-sterol reductase resulting in the release of NADP and 4,4-dimethyl-5a-cholesta-8,24-dien-3-b-ol. The latter reacts with hydrogen ion,oxygen and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-hydroxymethyl-4B-methyl-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with a hydrogen ion, water, and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-formyl-4b-methyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen, NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4B-methyl-4a-carboxy-cholesta-8,24-dien-3B-ol. The latter reacts with an NADP through c-3 sterol dehydrogenase resulting in the release of NADPH, carbon dioxide and 3-keto-4-methylzymosterol. The latter is reduced by NADPH through a 3-keto sterol reductase resulting in the release of NADP and 4a-methylzymosterol. The latter then reacts with hydrogen, oxygen and nadph through methylsterol monooxygenase resulting in the release of water, NADP and 4a-hydroxymethyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with water, hydrogen and NADPH through a methylsterol monooxygenase resulting in the release of water, NADP and 4a-formyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen and NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4a-carboxy-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with NADP through a C-3 sterol dehydrogenase resulting in the release of carbon dioxide, NADPH and 5a-cholesta-8,24-dien-3-one. The latter reacts with hydrogen ion and NADPH through a 3-keto sterol reductase resulting in the release of NADP and zymosterol. Zymosterol can either be used to create ergosterol starts with zymosterol reacting with S-adenosylmethionine through a sterol 24-c-methyltransferase resulting in the release of S-adenosylhomocysteine, hydrogen ion and fecosterol. Fecosterol reacts with C-8 sterol isomerase resulting in the release of episterol. Episterol reacts with oxygen, hydrogen ion and ferrocytochrome c through a C-5 sterol desaturase resulting in the release of ferricytochrome c, water and 5,7,24(28)-ergostatrienol. The latter reacts with hydrogen ion, oxygen, NADPH and c-22 sterol desaturase resulting in the release of water, NADP AND ERGOSTA-5,7,22,24(28)-tetraen-3-B-ol. The latter compound reacts with hydrogen ion and NADPH through a C-24 sterol reductase resulting in the release of NADP and ergosterol. Zymosterol reacts with C-8 sterol isomerase resulting in the release of 5a-cholesta-7,24-dien-3b-ol. The latter compound reacts with C-5 sterol desaturase resulting in the release of 7-dehydrodesmosterol. The latter is then converted spontaneously through desmosterol. Desmosterol is then spontaneously turned into cholesterol which can in turn react with tetradecanoyl-CoA spontaneously resulting in the release of Coenzyme A and CE(14:0).

The biosynthesis of Cholesterol starts with acetyl-CoA reacts with acetyl-CoA c-acetyltransferase resulting in the release of CoA acetoacetyl-CoA, The latter compound then reacts with an acetyl-coa through a hydroxymethylglutaryl-CoA synthase resulting in the release of 3-hydroxy-3-methylglutaryl-CoA. The latter compound in turn reacts with a NADPH through a 3-hydroxy-3-methylglutaryl-coenzyme A reductase resulting in the release of a NADP, Coenzyme A and Mevalonic acid. The latter is then phosphorylated by ATP through a mevalonate kinase resulting in the release of ADP and Mevalonic acid-5P which is then phosphorylated by ATP through a phosphomevalonate kinase resulting in the release of ADP and (S)-5-diphosphomevalonic acid. The latter compound in turn reacts with ATP through a diphosphomevalonic decarboxylase resulting in the release of phosphate, ADP, carbon dioxide and Isopentenyl pyrophosphate. The latter compound in turn reacts with isopentenyl diphosphate delta isomerase resulting in the release of dimethylallylpyrophosphate. The latter compound then reacts with isopentenyl pyrophosphate through a farnesyl pyrophosphate synthase resulting in the release of Geranyl-PP. The latter then reacts with an isopentenyl pyrophosphate through farnesyl pyrophosphate synthase resulting in the release of pyrophospate and farnesyl pyrophosphate. Farnesyl pyrophosphate then reacts with NADPH through a squalene synthase in order to produce squalene while also releasing two phosphates and NADP. Squalene then reacts with oxygen and NADPH through a squalene monooxygenase resulting in the release of water, NADP and (S)-2,3-epoxysqualene. The latter in turn reacts with lanosterol synthase resulting in the release of lanosterin. Lanosterin then reacts with oxygen and NADPH through a lanosterol 14-alpha demethylase resulting in the release of formic acid, water, NADP and 4,4-dimethylcholesta-8,14,24-trienol. The latter compound in turn is reduced by an NADPH through a Delta (14)-sterol reductase resulting in the release of NADP and 4,4-dimethyl-5a-cholesta-8,24-dien-3-b-ol. The latter reacts with hydrogen ion,oxygen and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-hydroxymethyl-4B-methyl-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with a hydrogen ion, water, and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-formyl-4b-methyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen, NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4B-methyl-4a-carboxy-cholesta-8,24-dien-3B-ol. The latter reacts with an NADP through c-3 sterol dehydrogenase resulting in the release of NADPH, carbon dioxide and 3-keto-4-methylzymosterol. The latter is reduced by NADPH through a 3-keto sterol reductase resulting in the release of NADP and 4a-methylzymosterol. The latter then reacts with hydrogen, oxygen and nadph through methylsterol monooxygenase resulting in the release of water, NADP and 4a-hydroxymethyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with water, hydrogen and NADPH through a methylsterol monooxygenase resulting in the release of water, NADP and 4a-formyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen and NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4a-carboxy-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with NADP through a C-3 sterol dehydrogenase resulting in the release of carbon dioxide, NADPH and 5a-cholesta-8,24-dien-3-one. The latter reacts with hydrogen ion and NADPH through a 3-keto sterol reductase resulting in the release of NADP and zymosterol. Zymosterol can either be used to create ergosterol starts with zymosterol reacting with S-adenosylmethionine through a sterol 24-c-methyltransferase resulting in the release of S-adenosylhomocysteine, hydrogen ion and fecosterol. Fecosterol reacts with C-8 sterol isomerase resulting in the release of episterol. Episterol reacts with oxygen, hydrogen ion and ferrocytochrome c through a C-5 sterol desaturase resulting in the release of ferricytochrome c, water and 5,7,24(28)-ergostatrienol. The latter reacts with hydrogen ion, oxygen, NADPH and c-22 sterol desaturase resulting in the release of water, NADP AND ERGOSTA-5,7,22,24(28)-tetraen-3-B-ol. The latter compound reacts with hydrogen ion and NADPH through a C-24 sterol reductase resulting in the release of NADP and ergosterol. Zymosterol reacts with C-8 sterol isomerase resulting in the release of 5a-cholesta-7,24-dien-3b-ol. The latter compound reacts with C-5 sterol desaturase resulting in the release of 7-dehydrodesmosterol. The latter is then converted spontaneously through desmosterol. Desmosterol is then spontaneously turned into cholesterol which can in turn react with hexanoyl-CoA spontaneously resulting in the release of Coenzyme A and CE(16:0).

The biosynthesis of Cholesterol starts with acetyl-CoA reacts with acetyl-CoA c-acetyltransferase resulting in the release of CoA acetoacetyl-CoA, The latter compound then reacts with an acetyl-coa through a hydroxymethylglutaryl-CoA synthase resulting in the release of 3-hydroxy-3-methylglutaryl-CoA. The latter compound in turn reacts with a NADPH through a 3-hydroxy-3-methylglutaryl-coenzyme A reductase resulting in the release of a NADP, Coenzyme A and Mevalonic acid. The latter is then phosphorylated by ATP through a mevalonate kinase resulting in the release of ADP and Mevalonic acid-5P which is then phosphorylated by ATP through a phosphomevalonate kinase resulting in the release of ADP and (S)-5-diphosphomevalonic acid. The latter compound in turn reacts with ATP through a diphosphomevalonic decarboxylase resulting in the release of phosphate, ADP, carbon dioxide and Isopentenyl pyrophosphate. The latter compound in turn reacts with isopentenyl diphosphate delta isomerase resulting in the release of dimethylallylpyrophosphate. The latter compound then reacts with isopentenyl pyrophosphate through a farnesyl pyrophosphate synthase resulting in the release of Geranyl-PP. The latter then reacts with an isopentenyl pyrophosphate through farnesyl pyrophosphate synthase resulting in the release of pyrophospate and farnesyl pyrophosphate. Farnesyl pyrophosphate then reacts with NADPH through a squalene synthase in order to produce squalene while also releasing two phosphates and NADP. Squalene then reacts with oxygen and NADPH through a squalene monooxygenase resulting in the release of water, NADP and (S)-2,3-epoxysqualene. The latter in turn reacts with lanosterol synthase resulting in the release of lanosterin. Lanosterin then reacts with oxygen and NADPH through a lanosterol 14-alpha demethylase resulting in the release of formic acid, water, NADP and 4,4-dimethylcholesta-8,14,24-trienol. The latter compound in turn is reduced by an NADPH through a Delta (14)-sterol reductase resulting in the release of NADP and 4,4-dimethyl-5a-cholesta-8,24-dien-3-b-ol. The latter reacts with hydrogen ion,oxygen and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-hydroxymethyl-4B-methyl-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with a hydrogen ion, water, and NADPH through a methylsterol monooxygenase resulting in the release of NADP, water and 4a-formyl-4b-methyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen, NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4B-methyl-4a-carboxy-cholesta-8,24-dien-3B-ol. The latter reacts with an NADP through c-3 sterol dehydrogenase resulting in the release of NADPH, carbon dioxide and 3-keto-4-methylzymosterol. The latter is reduced by NADPH through a 3-keto sterol reductase resulting in the release of NADP and 4a-methylzymosterol. The latter then reacts with hydrogen, oxygen and nadph through methylsterol monooxygenase resulting in the release of water, NADP and 4a-hydroxymethyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with water, hydrogen and NADPH through a methylsterol monooxygenase resulting in the release of water, NADP and 4a-formyl-5a-cholesta-8,24-dien-3B-ol. The latter reacts with oxygen and NADPH through methylsterol monooxygenase resulting in the release of water, NADP and 4a-carboxy-5a-cholesta-8,24-dien-3B-ol. The latter compound reacts with NADP through a C-3 sterol dehydrogenase resulting in the release of carbon dioxide, NADPH and 5a-cholesta-8,24-dien-3-one. The latter reacts with hydrogen ion and NADPH through a 3-keto sterol reductase resulting in the release of NADP and zymosterol. Zymosterol can either be used to create ergosterol starts with zymosterol reacting with S-adenosylmethionine through a sterol 24-c-methyltransferase resulting in the release of S-adenosylhomocysteine, hydrogen ion and fecosterol. Fecosterol reacts with C-8 sterol isomerase resulting in the release of episterol. Episterol reacts with oxygen, hydrogen ion and ferrocytochrome c through a C-5 sterol desaturase resulting in the release of ferricytochrome c, water and 5,7,24(28)-ergostatrienol. The latter reacts with hydrogen ion, oxygen, NADPH and c-22 sterol desaturase resulting in the release of water, NADP AND ERGOSTA-5,7,22,24(28)-tetraen-3-B-ol. The latter compound reacts with hydrogen ion and NADPH through a C-24 sterol reductase resulting in the release of NADP and ergosterol. Zymosterol reacts with C-8 sterol isomerase resulting in the release of 5a-cholesta-7,24-dien-3b-ol. The latter compound reacts with C-5 sterol desaturase resulting in the release of 7-dehydrodesmosterol. The latter is then converted spontaneously through desmosterol. Desmosterol is then spontaneously turned into cholesterol which can in turn react with stearidonoyl-CoA spontaneously resulting in the release of Coenzyme A and CE(18:0).

Lysosomal acid lipase deficiency, also called cholesteryl ester storage disease [CESD], LIPA deficiency or Wolman disease, is a rare autosomal recessive inborn error of metabolism. It's caused by not enough lysosomal acid lipase enzyme is produced at lysosome, which act to break down the fatty material. Lysosomal acid lipase enzyme catalyzes the conversion of cholesterol and fatty acid into cholesterol ester. This disorder is characterized by a large accumulation of cholesterol in the endoplasmic reticulum (ER). Symptoms of the disorder include vomiting, diarrhea, weight loss (i.e. feeding difficulties) and swelling of the abdomen. Lysosomal acid lipase deficiency can be treated with sebelipase alfa.

Lysosomal acid lipase deficiency, also called cholesteryl ester storage disease [CESD], LIPA deficiency or Wolman disease, is a rare autosomal recessive inborn error of metabolism. It's caused by not enough lysosomal acid lipase enzyme is produced at lysosome, which act to break down the fatty material. Lysosomal acid lipase enzyme catalyzes the conversion of cholesterol and fatty acid into cholesterol ester. This disorder is characterized by a large accumulation of cholesterol in the endoplasmic reticulum (ER). Symptoms of the disorder include vomiting, diarrhea, weight loss (i.e. feeding difficulties) and swelling of the abdomen. Lysosomal acid lipase deficiency can be treated with sebelipase alfa.

Lysosomal acid lipase deficiency, also called cholesteryl ester storage disease [CESD], LIPA deficiency or Wolman disease, is a rare autosomal recessive inborn error of metabolism. It's caused by not enough lysosomal acid lipase enzyme is produced at lysosome, which act to break down the fatty material. Lysosomal acid lipase enzyme catalyzes the conversion of cholesterol and fatty acid into cholesterol ester. This disorder is characterized by a large accumulation of cholesterol in the endoplasmic reticulum (ER). Symptoms of the disorder include vomiting, diarrhea, weight loss (i.e. feeding difficulties) and swelling of the abdomen. Lysosomal acid lipase deficiency can be treated with sebelipase alfa.

Cholestyramine is a bile acid sequestrant used as an adjunct in the reduction of elevated serum cholesterol in patients with primary hypercholesterolemia, and for the relief of pruritus associated with partial biliary obstruction. Cholesterol is probably the sole precursor of bile acids. During normal digestion, bile acids are secreted into the intestines. A major portion of the bile acids is absorbed from the intestinal tract and returned to the liver via the enterohepatic circulation. Only very small amounts of bile acids are found in normal serum. Cholestyramine resin adsorbs and combines with the bile acids in the intestine to form an insoluble complex which is excreted in the feces. This results in a partial removal of bile acids from the enterohepatic circulation by preventing their absorption. Cholestyramine resin is a strong anion exchange resin, allowing it to exchange its chloride anions with anionic bile acids present in the gastrointestinal tract and form a strong resin matrix. Since bile acid reabsorption and recycling is reduced, the liver has to synthesize new bile acid. This is done by increasing plasma LDL uptake. LDL is metabolized to form cholesterol. There is enhanced conversion of cholesterol to bile acids in the liver via 7a-hydroxylation. LDL receptors are upregulated in the liver to accommodate higher LDL uptake to replace the bile acids. Common adverse effects are dyspepsia and bloating. These symptoms can be decreased if cholestyramine is completely suspended in liquid several hours before ingestion. Constipation is a common side effect that is preventable with adequate water and fiber intake. Cholestyramine is considered a safe medication since it is not systemically absorbed.