Cholesterol biosynthesis pathway (WP197)

Homo sapiens

Cholesterol is a waxy steroid metabolite found in the cell membranes and transported in the blood plasma of all animals. It is an essential structural component of mammalian cell membranes, where it is required to establish proper membrane permeability and fluidity. In addition, cholesterol is an important component for the manufacture of bile acids, steroid hormones, and several fat-soluble vitamins. Cholesterol is the principal sterol synthesized by animals, but small quantities are synthesized in other eukaryotes, such as plants and fungi. It is almost completely absent among prokaryotes, which include bacteria. Source: [[wikipedia:Cholesterol|Wikipedia]] Proteins on this pathway have targeted assays available via the [ CPTAC Assay Portal]


Michael Lieberman , Patrick Ahles , Ned Mantei , Thomas Kelder , Samuel Sklar , Martijn Van Iersel , Egon Willighagen , Alex Pico , Bart Smeets , Daniel Riggins , Kristina Hanspers , Friederike Ehrhart , Denise Slenter , and Eric Weitz


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Homo sapiens



Pathway Ontology

cholesterol biosynthetic pathway


Label Type Compact URI Comment
(S)-2,3-Epoxysqualene Metabolite chebi:15441
Mevalonic acid Metabolite cas:150-97-0
Farnesyl pyrophosphate Metabolite cas:13058-04-3
Lathosterol Metabolite cas:80-99-9
Lanosterin Metabolite cas:79-63-0
Mevalonic acid 5-pyrophosphate Metabolite cas:1492-08-6
7-Dehydrocholesterol Metabolite cas:434-16-2
isopentenyl pyrophosphate Metabolite cas:358-71-4
HMG-CoA Metabolite hmdb:HMDB0001375
Geranyl-PP Metabolite cas:6699-20-3
Cholesterol Metabolite cas:57-88-5
Mevalonic acid-5P Metabolite cas:73566-35-5
Squalene Metabolite cas:111-02-4
Acetyl-CoA Metabolite cas:72-89-9
Dimethylallyl pyrophosphate Metabolite cas:358-72-5
NSDHL GeneProduct ncbigene:50814 NAD(P) dependent steroid dehydrogenase-like
FDPS GeneProduct ncbigene:2224
HMGCS1 GeneProduct ncbigene:3157
SC4MOL GeneProduct ncbigene:6307
SQLE GeneProduct ncbigene:6713
IDI1 GeneProduct ncbigene:3422
MVD GeneProduct ncbigene:4597
CYP51A1 GeneProduct ncbigene:1595
HMGCR GeneProduct ncbigene:3156
DHCR7 GeneProduct ncbigene:1717
PMVK GeneProduct ncbigene:10654
SC5DL GeneProduct ncbigene:6309
FDFT1 GeneProduct ncbigene:2222
LSS GeneProduct ncbigene:4047
MVK GeneProduct ncbigene:4598
FDPS GeneProduct ncbigene:2224


  1. Malformation syndromes due to inborn errors of cholesterol synthesis. Porter FD. J Clin Invest. 2002 Sep;110(6):715–24. PubMed Europe PMC Scholia