Animal models for disorders of hepatic lipoprotein metabolism |
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Authors: | T E Willnow J Herz |
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Institution: | (1) Department of Molecular Genetics, University of Texas Southwestern, 5323 Harry Hines Blvd., 75235 Dallas, TX, USA |
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Abstract: | The application of methods to create transgenic mice in which a gene of interest is either overexpressed or genetically inactivated has provided us with an ever-growing number of animal models to study complex physiological processes in vivo. Analysis of these mouse models has increased our knowledge about basic mechanisms that control biological systems and the pathological processes in human genetic disorders. This review focuses on the analysis of mouse models in which individual components of the hepatic clearance pathway for plasma lipoproteins have been inactivated. These studies have demonstrated that two hepatic lipoprotein receptors, the low-density lipoprotein receptor and the low-density lipoprotein receptor-related protein operate jointly in the uptake of dietary lipoproteins from the circulation. These findings have important implications for our understanding of pathophysiological processes resulting in hyperlipoproteinemia and atherosclerosis in patients.Abbreviations
apo
Apolipoprotein
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ER
Endoplasmic reticulum
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FPLC
Fast performance liquid chromatography
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FH
Familial hypercholesterolemia
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HDL
High-density lipoproteins
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IDL
Intermediate density lipoproteins
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LDL
Low-density lipoproteins
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LDLR LDL
Receptor
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LRP
LDLR-related protein
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RAP
Receptor-associated protein
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VLDL
Very low density lipoproteins |
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Keywords: | Low-density lipoprotein receptor Low-density lipoprotein receptor-related protein Chylomicron remnants Hyperlipoproteinemia Atherosclerosis |
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