The low-density lipoprotein receptor gene family encompasses a class of end
ocytic receptors that exhibit structural similarities to the low-density li
poprotein receptor. Members of this gene family are present in both vertebr
ate and nonvertebrate species. The identification of naturally occurring mu
tations and the application of gene targeting to inactivate receptor genes
enabled us to develop animal models to investigate the consequences of indi
vidual receptor defects in vivo. Analysis of these animal models revealed e
xciting new functions of lipoprotein receptors not only in systemic clearan
ce of lipoproteins but also in other important biological processes includi
ng reproduction, brain development, and adipositas.