KEGG   PATHWAY: etf04979
Entry
etf04979                    Pathway                                
Name
Cholesterol metabolism - Echinops telfairi (small Madagascar hedgehog)
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
Pathway map
etf04979  Cholesterol metabolism
etf04979

Other DBs
GO: 0008203
Organism
Echinops telfairi (small Madagascar hedgehog) [GN:etf]
Gene
101643958  ABCA1; phospholipid-transporting ATPase ABCA1 [KO:K05641]
101640718  APOA1; apolipoprotein A-I [KO:K08757]
101645959  LPL; lipoprotein lipase [KO:K01059] [EC:3.1.1.34]
101660554  LCAT; phosphatidylcholine-sterol acyltransferase [KO:K00650] [EC:2.3.1.43]
101650846  LIPG; endothelial lipase isoform X1 [KO:K22284] [EC:3.1.1.3]
101662295  phospholipid transfer protein [KO:K08761]
101658571  cholesteryl ester transfer protein [KO:K16835]
101643834  LIPC; hepatic triacylglycerol lipase isoform X1 [KO:K22283] [EC:3.1.1.3]
101659291  CD36; platelet glycoprotein 4 [KO:K06259]
101646129  LDLR; low-density lipoprotein receptor [KO:K12473]
101645303  NCEH1; neutral cholesterol ester hydrolase 1 isoform X1 [KO:K14349] [EC:3.1.1.-]
101639422  SOAT1; sterol O-acyltransferase 1 [KO:K00637] [EC:2.3.1.26]
101653803  SOAT2; sterol O-acyltransferase 2 [KO:K00637] [EC:2.3.1.26]
101659645  APOC2; apolipoprotein C-II [KO:K22287]
101663418  APOH; beta-2-glycoprotein 1 [KO:K17305]
101652997  ANGPTL3; angiopoietin-related protein 3 [KO:K22288]
101640030  ANGPTL4; angiopoietin-related protein 4 [KO:K08767]
101660529  ANGPTL8; angiopoietin-like protein 8 [KO:K22289]
101651490  SCARB1; scavenger receptor class B member 1 isoform X1 [KO:K13885]
101660326  SORT1; sortilin [KO:K12388]
101640837  APOB; apolipoprotein B-100 [KO:K14462]
101649994  PCSK9; LOW QUALITY PROTEIN: proprotein convertase subtilisin/kexin type 9 [KO:K13050] [EC:3.4.21.-]
101655546  LDLRAP1; low density lipoprotein receptor adapter protein 1 isoform X1 [KO:K12474]
101651321  E3 ubiquitin-protein ligase MYLIP [KO:K10637] [EC:2.3.2.27]
101642382  APOE; apolipoprotein E isoform X2 [KO:K04524]
101655513  LRP1; prolow-density lipoprotein receptor-related protein 1 isoform X2 [KO:K04550]
101642451  LRP2; low-density lipoprotein receptor-related protein 2 [KO:K06233]
101638537  LRPAP1; alpha-2-macroglobulin receptor-associated protein [KO:K22290]
101639718  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase isoform X2 [KO:K01052] [EC:3.1.1.13]
101638788  STARD3; stAR-related lipid transfer protein 3 [KO:K22291]
101656727  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
101653907  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
101649902  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
101642569  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 [KO:K15041]
101663436  TSPO2; translocator protein 2 [KO:K05770]
101660748  TSPO; translocator protein [KO:K05770]
101661290  sterol 26-hydroxylase, mitochondrial isoform X1 [KO:K00488] [EC:1.14.15.15]
101650077  VAPA; vesicle-associated membrane protein-associated protein A isoform X1 [KO:K06096]
101656452  VAPB; vesicle-associated membrane protein-associated protein B/C [KO:K10707]
101655971  NPC1; NPC intracellular cholesterol transporter 1 isoform X1 [KO:K12385]
101659365  OSBPL5; oxysterol-binding protein-related protein 5 isoform X3 [KO:K20464]
101652356  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
101647780  STAR; steroidogenic acute regulatory protein, mitochondrial [KO:K16931]
101644634  CIDEB; cell death activator CIDE-B [KO:K25914]
101638011  cytochrome P450 7A1 [KO:K00489] [EC:1.14.14.23]
101642968  ABCB11; LOW QUALITY PROTEIN: bile salt export pump [KO:K05664]
101648537  ABCG5; ATP-binding cassette sub-family G member 5 [KO:K05683]
101646124  ABCG8; ATP-binding cassette sub-family G member 8 isoform X1 [KO:K05684]
101645486  APOA2; apolipoprotein A-II [KO:K08758]
101661550  APOC3; apolipoprotein C-III [KO:K08759]
101661860  APOA4; apolipoprotein A-IV [KO:K08760]
Compound
C00162  Fatty acid
C00187  Cholesterol
C00422  Triacylglycerol
C00865  Phospholipid
C01921  Glycocholate
C02530  Cholesterol ester
C05122  Taurocholate
C05465  Taurochenodeoxycholate
C05466  Glycochenodeoxycholate
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
Lipids Health Dis 16:103 (2017)
DOI:10.1186/s12944-017-0488-4
Reference
  Authors
Oldoni F, Sinke RJ, Kuivenhoven JA
  Title
Mendelian disorders of high-density lipoprotein metabolism.
  Journal
Circ Res 114:124-42 (2014)
DOI:10.1161/CIRCRESAHA.113.300634
Reference
  Authors
Lusis AJ, Fogelman AM, Fonarow GC
  Title
Genetic basis of atherosclerosis: part I: new genes and pathways.
  Journal
Circulation 110:1868-73 (2004)
DOI:10.1161/01.CIR.0000143041.58692.CC
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
Nat Rev Drug Discov 7:84-99 (2008)
DOI:10.1038/nrd2353
Reference
  Authors
Ikonen E
  Title
Cellular cholesterol trafficking and compartmentalization.
  Journal
Nat Rev Mol Cell Biol 9:125-38 (2008)
DOI:10.1038/nrm2336
Reference
  Authors
Vaziri ND
  Title
HDL abnormalities in nephrotic syndrome and chronic kidney disease.
  Journal
Nat Rev Nephrol 12:37-47 (2016)
DOI:10.1038/nrneph.2015.180
Reference
  Authors
Kingwell BA, Chapman MJ, Kontush A, Miller NE
  Title
HDL-targeted therapies: progress, failures and future.
  Journal
Nat Rev Drug Discov 13:445-64 (2014)
DOI:10.1038/nrd4279
Reference
  Authors
Do RQ, Nicholls SJ, Schwartz GG
  Title
Evolving targets for lipid-modifying therapy.
  Journal
EMBO Mol Med 6:1215-30 (2014)
DOI:10.15252/emmm.201404000
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
Curr Opin Lipidol 26:56-63 (2015)
DOI:10.1097/MOL.0000000000000146
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
Circ J 74:2263-70 (2010)
DOI:10.1253/circj.CJ-10-0934
Reference
  Authors
Wang S, Smith JD
  Title
ABCA1 and nascent HDL biogenesis.
  Journal
Biofactors 40:547-54 (2014)
DOI:10.1002/biof.1187
Reference
  Authors
Hassan M
  Title
ANGPLT3: A novel modulator of lipid metabolism.
  Journal
Glob Cardiol Sci Pract 2017:e201706 (2017)
DOI:10.21542/gcsp.2017.6
Reference
  Authors
Dijk W, Kersten S
  Title
Regulation of lipoprotein lipase by Angptl4.
  Journal
Trends Endocrinol Metab 25:146-55 (2014)
DOI:10.1016/j.tem.2013.12.005
Reference
  Authors
Huuskonen J, Olkkonen VM, Jauhiainen M, Ehnholm C
  Title
The impact of phospholipid transfer protein (PLTP) on HDL metabolism.
  Journal
Atherosclerosis 155:269-81 (2001)
DOI:10.1016/S0021-9150(01)00447-6
Reference
  Authors
Shinkai H
  Title
Cholesteryl ester transfer-protein modulator and inhibitors and their potential for the treatment of cardiovascular diseases.
  Journal
Vasc Health Risk Manag 8:323-31 (2012)
DOI:10.2147/VHRM.S25238
Reference
  Authors
Norata GD, Tsimikas S, Pirillo A, Catapano AL
  Title
Apolipoprotein C-III: From Pathophysiology to Pharmacology.
  Journal
Trends Pharmacol Sci 36:675-87 (2015)
DOI:10.1016/j.tips.2015.07.001
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
Clin Chim Acta 460:11-7 (2016)
DOI:10.1016/j.cca.2016.06.013
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
Curr Opin Lipidol 23:213-9 (2012)
DOI:10.1097/MOL.0b013e3283532947
Reference
  Authors
Jiang W, Song BL
  Title
Ubiquitin ligases in cholesterol metabolism.
  Journal
Diabetes Metab J 38:171-80 (2014)
DOI:10.4093/dmj.2014.38.3.171
Reference
  Authors
Lambert G, Sjouke B, Choque B, Kastelein JJ, Hovingh GK
  Title
The PCSK9 decade.
  Journal
J Lipid Res 53:2515-24 (2012)
DOI:10.1194/jlr.R026658
Reference
  Authors
Graham A, Allen AM
  Title
Mitochondrial function and regulation of macrophage sterol metabolism and inflammatory responses.
  Journal
World J Cardiol 7:277-86 (2015)
DOI:10.4330/wjc.v7.i5.277
Reference
  Authors
Elustondo P, Martin LA, Karten B
  Title
Mitochondrial cholesterol import.
  Journal
Biochim Biophys Acta 1862:90-101 (2017)
DOI:10.1016/j.bbalip.2016.08.012
Reference
  Authors
Raiborg C, Wenzel EM, Stenmark H
  Title
ER-endosome contact sites: molecular compositions and functions.
  Journal
EMBO J 34:1848-58 (2015)
DOI:10.15252/embj.201591481
Reference
  Authors
van der Kant R, Neefjes J
  Title
Small regulators, major consequences - Ca(2)(+) and cholesterol at the endosome-ER interface.
  Journal
J Cell Sci 127:929-38 (2014)
DOI:10.1242/jcs.137539
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
Cell Metab 12:213-23 (2010)
DOI:10.1016/j.cmet.2010.08.006
Related
pathway
etf00100  Steroid biosynthesis
etf00120  Primary bile acid biosynthesis
etf00900  Terpenoid backbone biosynthesis
etf04975  Fat digestion and absorption
etf04976  Bile secretion
KO pathway
ko04979   
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