Indeed, 2 recent reports identified the critical role of EL in mediating LDL-C lowering by an LDLR independent pathway.10,11 These studies, in mice lacking both LDLR and EL, support the importance of the ANGPTL3/EL pathway in mediating VLDL remnant particle clearance and LDL-C lowering.10 The marked increase in IDL apoB FCR observed in our patients with hoFH after treatment with evinacumab are in line with those results. Here, ANGPTL3 is linked to homozygous familial hypercholesterolemia.