Purpose of reviewApolipoproteins and their receptors are the main controllers of lipid metabolism and, as such, have a major impact not only on the risk of cardiovascular disease but also on the development and degeneration of the central nervous system. Variations in the genes coding for these apolipoproteins and their receptors and the interaction with the environment determine individual susceptibility to metabolic disturbances, the response to dietary or pharmacological intervention and, finally, to disease.Recent findingsThis review will focus on recent findings, such as the latest concepts regarding apolipoprotein E in neurodevelopment, the newly identified apolipoprotein A-V and its influence in triglyceride metabolism, and the improved understanding of apolipoprotein A-I and HDL metabolism in the light of the discovery of the ABC family of transporters. Other key aspects of lipoprotein metabolism and cardiovascular disease risk such as apolipoprotein B-100, the LDL receptor, apolipoprotein C-III or apolipoprotein (a) will be updated.SummaryVariations in these genes will be analysed in relation to plasma lipid levels, their interactions with diet, treatment or other environmental stimuli, and their influence on the risk of cardiovascular disease and neurological disorders.