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10.1172/JCI29415 http://scihub22266oqcxt.onion/10.1172/JCI29415 17200716!1716209!17200716     free     free     free Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       J+Clin+Invest 2007 ; 117 (1): 165-74 Nephropedia Template TP {{tp|p=17200716|t=2007. Disruption of LDL but not VLDL clearance in autosomal recessive hypercholesterolemia |pdf=|usr=}}{{17200716}} gab.com Text Disruption of LDL but not VLDL clearance in autosomal recessive hypercholesterolemia JO: J Clin Invest http://www.kidney.de/pget.php?&tw=1&pmid=17200716 #FOAvip 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. Twit Text FOAVip Disruption of LDL but not VLDL clearance in autosomal recessive hypercholesterolemia ????J Clin Invest http://www.kidney.de/pget.php?&tw=1&pmid=17200716 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=17200716 #FOAvip English Wikipedia <ref>{{Cite pmid|17200716}}</ref> Disruption of LDL but not VLDL clearance in autosomal recessive hypercholesterolemia #MMPMID17200716 Jones C; Garuti R; Michaely P; Li WP; Maeda N; Cohen JC; Herz J; Hobbs HH J Clin Invest 2007[Jan]; 117 (1): 165-74 PMID17200716show ga 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. |Animals[MESH] |Cholesterol/blood/metabolism[MESH] |Genes, Recessive[MESH] |Hyperlipoproteinemia Type II/blood/*genetics/metabolism[MESH] |Lipoproteins, LDL/*blood[MESH] |Lipoproteins, VLDL/*blood/metabolism[MESH] |Lipoproteins/blood/metabolism[MESH] |Liver/metabolism[MESH] |Mice[MESH] |Mice, Knockout[MESH]Disruption of LDL but not VLDL clearance in autosomal recessive hyper(epmc).pdf DeepDyve Pubget Overpricing