It is known that, in the general human population, serum
fatty acid composition is correlated with serum
triacylglycerol and
cholesterol concentrations. The goal of the present study was to analyze whether the same is true of individuals who have a
low density lipoprotein receptor (
LDL-R) defect. Concentrations of 16 different
fatty acids,
cholesterol,
triacylglycerol, and major
lipoproteins in serum were determined in eight individuals who had (FH-North Karelia), the most common
LDL-R defect in Finland, which causes
familial hypercholesterolemia, and in their 30 relatives belonging to a single large pedigree as controls. The average number of double bonds (i.e., degree of desaturation) in serum
fatty acids correlated negatively with the concentrations of serum total
cholesterol (r = 0.27, P < 0.05) and total
triacylglycerol (r = -0.71, P < 0.001) and positively with the number of fish meals per week (r = 0.50, P < 0.01), which was analyzed in all pedigree members jointly. These effects were similar in individuals having
LDL-R defect, in which group the correlation coefficients were -0.31 (P = NS), -0.99 (P < 0.001), and 0.79 (P = NS) for serum total
cholesterol,
triacylglycerol, and weekly fish meals, respectively. Thus,
LDL-R defect does not impair the correlation between serum
fatty acid composition and serum
triacylglycerol concentration. This result is in agreement with dietary studies that have shown that
familial hypercholesterolemia patients respond very favorably to dietary
therapy.