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A mouse model of human familial hypercholesterolemia: Markedly elevated low density lipoprotein cholesterol levels and severe atherosclerosis on a low-fat chow diet
Abstract
Mutations in the low density lipoprotein (LDL) receptor gene cause familial hypercholesterolemia, a human disease characterized by premature atherosclerosis and markedly elevated plasma levels of LDL cholesterol and apolipoprotein (apo) B100. In contrast, mice deficient for the LDL receptor (Ldlr-/-) have only mildly elevated LDL cholesterol levels and little atherosclerosis. This difference results from extensive editing of the hepatic apoB mRNA in the mouse, which limits apoB100 synthesis in favor of apoB48 synthesis. We have generated Ldlr-/- mice that cannot edit the apoB mRNA and therefore synthesize exclusively apoB100. These mice had markedly elevated LDL cholesterol and apoB100 levels and developed extensive atherosclerosis on a chow diet. This authentic model of human familial hypercholesterolemia will provide a new tool for studying atherosclerosis.
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The apoB100/LDLR-/- mouse mimics human familial hypercholesterolaemia.
“The apoB100/LDLR-/- mouse developed by Powell-Braxton et al. [13] mimics FH very well since the hypercholesterolaemia these animals suffer is caused almost entirely by high plasma concentrations of cholesterol-rich apoB100-containing LDL, i.e., the same as that which typically occurs in humans.”
From ApoB100/LDLR-/- Hypercholesterolaemic Mice as a Model for Mild Cognitive Impairment and Neuronal Damage (2011) · cites this paper as [13]
The hypercholesterolaemia in apoB100/LDLR-/- mice is caused almost entirely by high plasma concentrations of cholesterol-rich apoB100-containing LDL.
“The apoB100/LDLR-/- mouse developed by Powell-Braxton et al. [13] mimics FH very well since the hypercholesterolaemia these animals suffer is caused almost entirely by high plasma concentrations of cholesterol-rich apoB100-containing LDL, i.e., the same as that which typically occurs in humans.”
From ApoB100/LDLR-/- Hypercholesterolaemic Mice as a Model for Mild Cognitive Impairment and Neuronal Damage (2011) · cites this paper as [13]
The transgenic mice have markedly elevated LDL cholesterol and apoB100 levels, providing a model of human FH, and develop extensive atherosclerosis when fed a chow diet.
“This results in markedly elevated LDL cholesterol and apoB100 levels (thus providing a model of human FH) that develop extensive atherosclerosis when fed a chow diet [13].”
From ApoB100/LDLR-/- Hypercholesterolaemic Mice as a Model for Mild Cognitive Impairment and Neuronal Damage (2011) · cites this paper as [13]
The transgenic mice used in the present study are characterized by their deficiency in LDLRs and in their inability to splice apoB mRNA.
“The transgenic mice used in the present study are characterized by their deficiency in LDLRs and in their inability to splice apoB mRNA.”
From ApoB100/LDLR-/- Hypercholesterolaemic Mice as a Model for Mild Cognitive Impairment and Neuronal Damage (2011) · cites this paper as [13]
Male mice show higher levels of total and LDL-cholesterol than female mice.
“Male mice were selected because they show higher levels of total and LDL-cholesterol than females [13].”
From ApoB100/LDLR-/- Hypercholesterolaemic Mice as a Model for Mild Cognitive Impairment and Neuronal Damage (2011) · cites this paper as [13]
From birth, apoB100/LDLR-/- mice have markedly elevated LDL-cholesterol and apolipoprotein B100 levels.
“From birth, these animals have markedly elevated LDL-cholesterol and apolipoprotein B100 (apoB100) levels.”
From ApoB100/LDLR-/- Hypercholesterolaemic Mice as a Model for Mild Cognitive Impairment and Neuronal Damage (2011) · cites this paper as [13]
Citations
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Bertrand, N., Grenier, P., Mahmoudi, M., Lima, E. M., Appel, E. A., Dormont, F., Lim, J.-M., Karnik, R., Langer, R., & Farokhzad, O. C. (2017) · Nature Communications · doi:10.1038/s41467-017-00600-w · Cited by 708
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Tsang, H. G., Rashdan, N. A., Whitelaw, C. B. A., Corcoran, B. M., Summers, K. M., & MacRae, V. E. (2016) · Cell Biochemistry and Function · doi:10.1002/cbf.3173 · Cited by 154
Smallwood, T. L., Gatti, D. M., Quizon, P., Weinstock, G. M., Jung, K.-C., Zhao, L., Hua, K., Pomp, D., & Bennett, B. J. (2014) · G3 Genes|Genomes|Genetics · doi:10.1534/g3.114.014704 · Cited by 54
Young, S., Veniant, M., Beigneux, A., Bensadoun, A., & Fong, L. (2008) · Current Drug Targets · doi:10.2174/138945008783755629 · Cited by 33
Self-citationRamírez, C., Sierra, S., Tercero, I., Vázquez, J. A., Pineda, A., Manrique, T., & Burgos, J. S. (2011) · PLoS ONE · doi:10.1371/journal.pone.0022712 · Cited by 31
Metadata
- Authors
- Powell-Braxton, L., Véniant, M., Latvala, R. D., Hirano, K.-I., Won, W. B., Ross, J., Dybdal, N., Zlot, C. H., Young, S. G., & Davidson, N. O.
- Venue
- Nature Medicine
- Published
- 1998-08-01
- Volume
- 4
- Issue
- 8
- Pages
- 934-938
- ISSN
- 1078-8956
- 1546-170X
- Cited by
- 192
- References
- 3
- Language
- en
- Update notices
- erratum (1998-10-01)
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