1984 年 11 巻 6 号 p. 1255-1260
Plasma lipoprotein abnormalities in patients with two different types of familial lecithin: cholesterol acyltransferase (LCAT) deficiency, “classical” type and “partial” type were studied. The apoprotein composition in lipoprotein fractions were similar in both types, but the levels of apoprotein were different. Plasma apo A I and A II levels were low in both types, but the decrease was prominent in “classical” type. Plasma apo B 100 level remained within the normal range in “partial” type but it was low in “classical” type. The levels of apo B 48 in VLDL from “partial” type increased slightly and apo B 48 level in VLDL from “classical” type was very high.
The content of discoidal HDL was low and the content of small spherical HDL was high in “partial” type as compaired with those in “classical” type. The content of large sized LDL was high in “classical” type and that was low in “partial” type. The large-sized-LDL contained significant amount of apo B 48, and the increase in the content of large-sized LDL may be related to the pathogenesis of renal injury in patients with “classical” type.