A mouse model with features of familial combined hyperlipidemia.
Familial combined hyperlipidemia (FCHL) is a common inherited lipid disorder, affecting 1 to 2 percent of the population in Westernized societies. Individuals with FCHL have large quantities of very low density lipoprotein (VLDL) and low density lipoprotein (LDL) and develop premature coronary heart disease. A mouse model displaying some of the features of FCHL was created by crossing mice carrying the human apolipoprotein C-III (APOC3) transgene with mice deficient in the LDL receptor. A synergistic interaction between the apolipoprotein C-III and the LDL receptor defects produced large quantities of VLDL and LDL and enhanced the development of atherosclerosis. This mouse model may provide clues to the origin of human FCHL.
Masucci-Magoulas
L
L
Department of Medicine, Columbia University, 630 West 168 Street, New York, NY 10032, USA.
Goldberg
I J
IJ
Bisgaier
C L
CL
Serajuddin
H
H
Francone
O L
OL
Breslow
J L
JL
Tall
A R
AR
D000818:Animals; D053305:Apolipoprotein C-III; D001055:Apolipoproteins B / Q000097:blood; D001056:Apolipoproteins C / Q000235:genetics*; D001057:Apolipoproteins E / Q000097:blood; D001161:Arteriosclerosis / Q000209:etiology; D002352:Carrier Proteins / Q000235:genetics; D002784:Cholesterol / Q000097:blood; D053480:Cholesterol Ester Transfer Proteins; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D015243:Cholesterol, VLDL / Q000097:blood; D004032:Diet; D004195:Disease Models, Animal; D004198:Disease Susceptibility; D005260:Female; D006023:Glycoproteins; D006801:Humans; D006950:Hyperlipidemia, Familial Combined / Q000097:blood / Q000235:genetics; D006953:Hyperlipoproteinemia Type IV / Q000235:genetics; D008074:Lipoproteins / Q000097:blood; D008079:Lipoproteins, VLDL / Q000097:blood; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D011973:Receptors, LDL / Q000235:genetics* / Q000378:metabolism; D019076:Transgenes; D014280:Triglycerides / Q000097:blood
D053305:Apolipoprotein C-III; D001055:Apolipoproteins B; D001056:Apolipoproteins C; D001057:Apolipoproteins E; C506642:CETP protein, human; D002352:Carrier Proteins; D053480:Cholesterol Ester Transfer Proteins; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D015243:Cholesterol, VLDL; D006023:Glycoproteins; D008074:Lipoproteins; D008079:Lipoproteins, VLDL; D011973:Receptors, LDL; D014280:Triglycerides; C030104:lipoprotein cholesterol; D002784:Cholesterol