Metabolism of low-density lipoproteins by cultured hepatocytes from normal and homozygous familial hypercholesterolemic subjects [PDF]
The profoundly elevated concentrations of low-density lipoproteins (LDL) present in homozygous familial hypercholesterolemia lead to symptomatic cardiovascular disease and death by early adulthood.
Brewer, HB+6 more
core +1 more source
LDL Receptors in Keratinocytes
The presence of sufficient amounts of cholesterol in the epidermis is necessary for proper functioning of plasma membranes in the viable epidermal cell layers and also for the barrier quality of lipid intercellular bilayers of the stratum corneum. Cholesterol can be generated by local epidermal synthesis, or imported from the circulation as low-density
Maria Ponec+5 more
openaire +3 more sources
Mechanisms of dysregulation of low-density lipoprotein receptor expression in vascular smooth muscle cells by inflammatory cytokines [PDF]
Objective - Although inflammation is a recognized feature of atherosclerosis, the impact of inflammation on cellular cholesterol homeostasis is unclear.
Ma, KL+6 more
core +1 more source
Elimination of amyloid-ss peptide (Ass) from the brain is poorly understood. After intracerebral microinjections in young mice, (125)I-Ass(1-40) was rapidly removed from the brain (t(1/2)
Masayoshi Shibata+10 more
semanticscholar +1 more source
Liver Transplantation to Provide Low-Density-Lipoprotein Receptors and Lower Plasma Cholesterol in a Child with Homozygous Familial Hypercholesterolemia [PDF]
A six-year-old girl with severe hypercholesterolemia and atherosclerosis had two defective genes at the low-density-lipoprotein (LDL) receptor locus, as determined by biochemical studies of cultured fibroblasts.
Cummings+9 more
core +1 more source
A common polymorphism in the LDL receptor gene has multiple effects on LDL receptor function [PDF]
A common synonymous single nucleotide polymorphism in exon 12 of the low-density lipoprotein receptor (LDLR) gene, rs688, has been associated with increased plasma total and LDL cholesterol in several populations. Using immortalized lymphoblastoid cell lines from a healthy study population, we confirmed an earlier report that the minor allele of rs688 ...
Gao, Feng+3 more
openaire +5 more sources
Molecular genetics of the LDL receptor gene in familial hypercholesterolemia
The low density lipoprotein (LDL) receptor is a cell surface transmembrane protein that mediates the uptake and lysosomal degradation of plasma LDL, thereby providing cholesterol to cells.
H. Hobbs, Michael S. Brown, J. Goldstein
semanticscholar +1 more source
Coaxing the LDL Receptor Family into the Fold [PDF]
Low-density-lipoprotein (LDL) receptor family members control diverse developmental and physiological pathways. In this issue of Cell, both Culi and Mann and Hsieh et al. report on Boca/MESD, a highly conserved chaperone required for transport of LDLR family proteins to the cell surface.
Joachim Herz, Peter Marschang
openaire +3 more sources
Structural basis for the recognition of LDL-receptor family members by VSV glycoprotein
Vesicular stomatitis virus (VSV) is an oncolytic rhabdovirus and its glycoprotein G is widely used to pseudotype other viruses for gene therapy. Low-density lipoprotein receptor (LDL-R) serves as a major entry receptor for VSV. Here we report two crystal
Jovan Nikolic+5 more
semanticscholar +1 more source
Lipoprotein assembly and function in an evolutionary perspective
Circulatory fat transport in animals relies on members of the large lipid transfer protein (LLTP) superfamily, including mammalian apolipoprotein B (apoB) and insect apolipophorin II/I (apoLp-II/I).
Van der Horst Dick J., Rodenburg Kees W.
doaj +1 more source