ArticleThe Journal of clinical investigation2007
Disruption of LDL but not VLDL clearance in autosomal recessive hypercholesterolemia.
Article in The Journal of clinical investigation, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
29 citing papers in PubMed, 59 citations in OpenAlex.
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- Endothelial lipase facilitates low-density lipoprotein (LDL) uptake in LDL receptor deficiency by a heparan sulfate proteoglycan-dependent mechanism.bioRxiv : the preprint server for biology · 2025Article
- Glucagon receptor signaling at white adipose tissue does not regulate lipolysis.American journal of physiology. Endocrinology and metabolism · 2022Article
- Deletion of LDLRAP1 Induces Atherosclerotic Plaque Formation, Insulin Resistance, and Dysregulated Insulin Response in Adipose Tissue.The American journal of pathology · 2022Article
- GALNT2 regulates ANGPTL3 cleavage in cells and in vivo of mice.Scientific reports · 2020Article
- Science, serendipity, and the single degree.The Journal of clinical investigation · 2018Article
- Inactivation of Tm6sf2, a Gene Defective in Fatty Liver Disease, Impairs Lipidation but Not Secretion of Very Low Density Lipoproteins.The Journal of biological chemistry · 2016Article
- Endocytic adaptors Arh and Dab2 control homeostasis of circulatory cholesterol.Journal of lipid research · 2016Article
- CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL.Nature communications · 2016Article
- Endocytosis and Physiology: Insights from Disabled-2 Deficient Mice.Frontiers in cell and developmental biology · 2016Review
- Review
- Hyperlipoproteinemia type 3: the forgotten phenotype.Current atherosclerosis reports · 2014Review
- Current perspectives in genetic cardiovascular disorders: from basic to clinical aspects.Heart and vessels · 2014Review
- S-nitrosylation of ARH is required for LDL uptake by the LDL receptor.Journal of lipid research · 2013Article
- Chronic overexpression of PNPLA3I148M in mouse liver causes hepatic steatosis.The Journal of clinical investigation · 2012Article
- Low-density lipoprotein receptor (LDLR) family orchestrates cholesterol homeostasis.The Yale journal of biology and medicine · 2012Review
- Mechanisms and genetic determinants regulating sterol absorption, circulating LDL levels, and sterol elimination: implications for classification and disease risk.Journal of lipid research · 2011Review
- The metabolism of triglyceride-rich lipoproteins revisited: new players, new insight.Atherosclerosis · 2010Review
- Enhanced hepatic apoA-I secretion and peripheral efflux of cholesterol and phospholipid in CD36 null mice.PloS one · 2010Article
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
Abstract
Genetic defects in LDL clearance result in severe hypercholesterolemia and premature atherosclerosis. Mutations in the LDL receptor (LDLR) cause familial hypercholesterolemia (FH), the most severe form of genetic hypercholesterolemia. A phenocopy of FH, autosomal recessive hypercholesterolemia (ARH), is due to mutations in an adaptor protein involved in LDLR internalization. Despite comparable reductions in LDL clearance rates, plasma LDL levels are substantially lower in ARH than in FH. To determine the metabolic basis for this difference, we examined the synthesis and catabolism of VLDL in murine models of FH (Ldlr(-/-)) and ARH (Arh(-/-)). The hyperlipidemic response to a high-sucrose diet was greatly attenuated in Arh(-/-) mice compared with Ldlr(-/-) mice despite similar rates of VLDL secretion. The rate of VLDL clearance was significantly higher in Arh(-/-) mice than in Ldlr(-/-) mice, suggesting that LDLR-dependent uptake of VLDL is maintained in the absence of ARH. Consistent with these findings, hepatocytes from Arh(-/-) mice (but not Ldlr(-/-) mice) internalized beta-migrating VLDL (beta-VLDL). These results demonstrate that ARH is not required for LDLR-dependent uptake of VLDL by the liver. The preservation of VLDL remnant clearance attenuates the phenotype of ARH and likely contributes to greater responsiveness to statins in ARH compared with FH.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.