Markedly Accelerated Catabolism of Apolipoprotein a-Ii (Apoa-Ii) and HighDensity-Lipoproteins Containing Apoa-Ii

Classic (complete) lecithin:cholesterol acyltransferase (LCAT) deficiency and Fish-eye disease (partial LCAT deficiency) are genetic syndromes associated with markedly decreased plasma levels of high density lipoprotein (HDL) cholesterol but not with an increased risk of atherosclerotic cardiovascular dis-ease. We investigated the metabolism of the HDL apolipopro-teins (apo) apoA-I and apoA-II in a total of five patients with LCAT deficiency, one with classic LCAT deficiency and four with Fish-eye disease. Plasma levels ofapoA-II were decreased to a proportionately greater extent (23 % ofnormal) than apoA-1 (30 % of normal). In addition, plasma concentrations ofHDL particles containing both apoA-I and apoA-II (LpA-I:A-II) were much lower (18 % of normal) than those of particles con-taining only apoA-I (LpA-I) (51 % of normal). The metaboli