Entry
Name
Cholesterol metabolism
Description
Cholesterol is an essential component of mammalian cell membranes as well as a precursor of bile acids, vitamin D and steroid hormones. Cholesterol homeostasis in humans is regulated by well-balanced mechanisms of intestinal uptake, endogenous synthesis, transport in lipoprotein particles, and biliary excretion. Its abnormal metabolism can lead to increased risk for various endocrine disorders and cardiovascular diseases.
Class
Organismal Systems; Digestive system
BRITE hierarchy
Pathway map
Other DBs
Orthology
K01052 LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [EC:3.1.1.13 ]
K04550 LRP1, CD91; low-density lipoprotein receptor-related protein 1 (alpha-2-macroglobulin receptor)
K05641 ABCA1; ATP-binding cassette, subfamily A (ABC1), member 1
K05664 ABCB11; ATP-binding cassette, subfamily B (MDR/TAP), member 11
K05683 ABCG5; ATP-binding cassette, subfamily G (WHITE), member 5 (sterolin 1)
K05684 ABCG8; ATP-binding cassette, subfamily G (WHITE), member 8 (sterolin 2)
K05770 TSPO, BZRP; translocator protein
K05862 VDAC1; voltage-dependent anion channel protein 1
K06096 VAPA; vesicle-associated membrane protein-associated protein A
K06233 LRP2; low density lipoprotein-related protein 2
K08757 APOA1; apolipoprotein A-I
K08758 APOA2; apolipoprotein A-II
K08759 APOC3; apolipoprotein C-III
K08760 APOA4; apolipoprotein A-IV
K08761 PLTP; phospholipid transfer protein
K08767 ANGPTL4, PGAR; angiopoietin-like 4
K09644 LPA; apolipoprotein(a) [EC:3.4.21.-]
K10707 VAPB, ALS8; vesicle-associated membrane protein-associated protein B
K12385 NPC1; Niemann-Pick C1 protein
K12473 LDLR; low-density lipoprotein receptor
K12474 LDLRAP1, ARH; low density lipoprotein receptor adapter protein 1
K13050 PCSK9; proprotein convertase subtilisin/kexin type 9 [EC:3.4.21.-]
K13443 NPC2; Niemann-Pick C2 protein
K13885 SCARB1; scavenger receptor class B, member 1
K14349 NCEH1, AADACL1; neutral cholesterol ester hydrolase 1 [EC:3.1.1.-]
K15040 VDAC2; voltage-dependent anion channel protein 2
K15041 VDAC3; voltage-dependent anion channel protein 3
K16835 CETP; cholesteryl ester transfer protein
K16931 STAR; steroidogenic acute regulatory protein, mitochondrial
K17305 APOH, B2G1; beta-2-glycoprotein 1
K20464 OSBPL5, ORP5; oxysterol-binding protein-related protein 5
K22286 APOC1; apolipoprotein C-I
K22287 APOC2; apolipoprotein C-II
K22288 ANGPTL3; angiopoietin-like 3
K22289 ANGPTL8, RIFL; angiopoietin-like 8
K22290 LRPAP1; alpha-2-macroglobulin receptor-associated protein
K22291 STARD3; StAR-related lipid transfer protein 3
K25914 CIDEB; cell death activator CIDE-B
Compound
C15610 Cholest-5-ene-3beta,26-diol
Reference
Authors
Paththinige CS, Sirisena ND, Dissanayake V
Title
Genetic determinants of inherited susceptibility to hypercholesterolemia - a comprehensive literature review.
Journal
Reference
Authors
Oldoni F, Sinke RJ, Kuivenhoven JA
Title
Mendelian disorders of high-density lipoprotein metabolism.
Journal
Reference
Authors
Lusis AJ, Fogelman AM, Fonarow GC
Title
Genetic basis of atherosclerosis: part I: new genes and pathways.
Journal
Reference
Authors
Wasan KM, Brocks DR, Lee SD, Sachs-Barrable K, Thornton SJ
Title
Impact of lipoproteins on the biological activity and disposition of hydrophobic drugs: implications for drug discovery.
Journal
Reference
Authors
Ikonen E
Title
Cellular cholesterol trafficking and compartmentalization.
Journal
Reference
Authors
Vaziri ND
Title
HDL abnormalities in nephrotic syndrome and chronic kidney disease.
Journal
Reference
Authors
Kingwell BA, Chapman MJ, Kontush A, Miller NE
Title
HDL-targeted therapies: progress, failures and future.
Journal
Reference
Authors
Do RQ, Nicholls SJ, Schwartz GG
Title
Evolving targets for lipid-modifying therapy.
Journal
Reference
Authors
Feingold KR, Grunfeld C
Title
Introduction to Lipids and Lipoproteins
Journal
Endotext (2000)
Reference
Authors
Sacks FM
Title
The crucial roles of apolipoproteins E and C-III in apoB lipoprotein metabolism in normolipidemia and hypertriglyceridemia.
Journal
Reference
Authors
Yasuda T, Ishida T, Rader DJ
Title
Update on the role of endothelial lipase in high-density lipoprotein metabolism, reverse cholesterol transport, and atherosclerosis.
Journal
Reference
Authors
Wang S, Smith JD
Title
ABCA1 and nascent HDL biogenesis.
Journal
Reference
Authors
Hassan M
Title
ANGPLT3: A novel modulator of lipid metabolism.
Journal
Reference
Authors
Dijk W, Kersten S
Title
Regulation of lipoprotein lipase by Angptl4.
Journal
Reference
Authors
Huuskonen J, Olkkonen VM, Jauhiainen M, Ehnholm C
Title
The impact of phospholipid transfer protein (PLTP) on HDL metabolism.
Journal
Reference
Authors
Shinkai H
Title
Cholesteryl ester transfer-protein modulator and inhibitors and their potential for the treatment of cardiovascular diseases.
Journal
Reference
Authors
Norata GD, Tsimikas S, Pirillo A, Catapano AL
Title
Apolipoprotein C-III: From Pathophysiology to Pharmacology.
Journal
Reference
Authors
Zhong LY, Cayabyab FS, Tang CK, Zheng XL, Peng TH, Lv YC
Title
Sortilin: A novel regulator in lipid metabolism and atherogenesis.
Journal
Reference
Authors
Sorrentino V, Zelcer N
Title
Post-transcriptional regulation of lipoprotein receptors by the E3-ubiquitin ligase inducible degrader of the low-density lipoprotein receptor.
Journal
Reference
Authors
Jiang W, Song BL
Title
Ubiquitin ligases in cholesterol metabolism.
Journal
Reference
Authors
Lambert G, Sjouke B, Choque B, Kastelein JJ, Hovingh GK
Title
The PCSK9 decade.
Journal
Reference
Authors
Graham A, Allen AM
Title
Mitochondrial function and regulation of macrophage sterol metabolism and inflammatory responses.
Journal
Reference
Authors
Elustondo P, Martin LA, Karten B
Title
Mitochondrial cholesterol import.
Journal
Reference
Authors
Raiborg C, Wenzel EM, Stenmark H
Title
ER-endosome contact sites: molecular compositions and functions.
Journal
Reference
Authors
van der Kant R, Neefjes J
Title
Small regulators, major consequences - Ca(2)(+) and cholesterol at the endosome-ER interface.
Journal
Reference
Authors
Kjolby M, Andersen OM, Breiderhoff T, Fjorback AW, Pedersen KM, Madsen P, Jansen P, Heeren J, Willnow TE, Nykjaer A
Title
Sort1, encoded by the cardiovascular risk locus 1p13.3, is a regulator of hepatic lipoprotein export.
Journal
Related pathway
ko00120 Primary bile acid biosynthesis
ko00900 Terpenoid backbone biosynthesis
ko04975 Fat digestion and absorption