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Skin fibroblasts from a patient with homozygous familial hypercholesterolemia (HFH) were compared with normal skin fibroblasts with regard to binding, internalization, and degradation of iodinated human low density lipoprotein (LDL). Like other cell lines from HFH patients, the mutant cells showed no suppression of sterol synthesis by LDL. Surface binding, measured at 0 degrees to eliminate the appreciable internalization that was shown to occur at 37 degrees, was on the average slightly less for HFH cells than normal cells at low LDL concentrations but comparable or even greater at high LDL concentrations (greater than 60 mug of LDL protein per ml). A major defect observed was in the rate of internalization of LDL at 37 degrees, which was only 1-10% of that in normal cells. LDL degradation was also markedly reduced but not to the same extent. Thus, a larger fraction of the LDL taken up appeared to be degraded by the mutant cells. The most striking defect observed, then, was not in surface binding of LDL but in rate of LDL internalization. While this might be secondary to a defect in specific binding sites of LDL, the magnitude of the observed differences in binding at low temperature seems too small to account for the huge differences in internalization (13- to 115-fold).

proceedings of the national academy of sciences of the united states of america

Binding, internalization, and degradation of low density lipoprotein by normal human fibroblasts and by fibroblasts from a case of homozygous familial hypercholesterolemia.