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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 |
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| Author(s): Powell-Braxton L, Veniant M, Latvala RD, Hirano KI, Won WB, Ross J, Dybdal N, Zlot CH, Young SG, Davidson NO |
| Source: NATURE MEDICINE Volume: 4 Issue: 8 Pages: 934-938 Published: AUG 1998 |
| Times Cited: 74 References: 33 |
| 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 familiar hypercholesterolemia will provide a new tool for studying atherosclerosis. |
| Document Type: Article |
| Language: English |
| Reprint Address: Davidson, NO (reprint author), Washington Univ, Sch Med, Dept Med, St Louis, MO 63110 USA |
Addresses:
1. Genentech Inc, Cardiovasc Res, S San Francisco, CA 94080 USA
2. Univ Calif San Francisco, Gladstone Inst Cardiovasc Dis, Dept Med, San Francisco, CA 94141 USA
3. Univ Calif San Francisco, Cardiovasc Res Inst, San Francisco, CA 94141 USA
4. Univ Chicago, Dept Med, Chicago, IL 60637 USA |
| Publisher: NATURE AMERICA INC, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 USA |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology; Medicine, Research & Experimental |
| IDS Number: 106AK |
| ISSN: 1078-8956 |
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