Journal of Lipid Research, Vol 21, 1116-1127, Copyright © 1980 by Lipid Research, Inc.
Abnormalities in lipoproteins of d < 1.006 g/ml in familial lecithin:cholesterol acyltransferase deficiency
JA Glomset, K Applegate, T Forte, WC King, CD Mitchell, KR Norum and E Gjone
Studies of different sized lipoproteins of d < 1.006 g/ml from patients
with familial lecithin:cholesterol acyltransferase deficiency have yielded
new evidence of abnormalities in this lipoprotein class. Lipoproteins of
all sizes contain high amounts of unesterified cholesterol, low amounts of
total protein, and particularly low amounts of apolipoproteins C-II and
C-III. Lipoproteins 60 nm in diameter or larger include particles that show
a notched appearance upon electron microscopy, and contain a) a high
combined volume of phospholipid, unesterified cholesterol, and protein; b)
high amounts of cholesteryl ester and apolipoproteins C-I and E, and c) two
major tetramethylurea- insoluble proteins that can be separated by
electrophoresis in the presence of sodium dodecylsulfate. In contrast,
lipoproteins that are 40 nm in diameter or less appear to contain low
amounts of cholesteryl ester, normal amounts of apolipoproteins C-I and E,
and a single tetramethylurea-insoluble protein the size of that in control
lipoproteins. Since these abnormalities occur in the lipoproteins of four
different patients from four different families, they are probably effects
of the enzyme deficiency. Most, however, appear to arise indirectly because
in vitro experiments published earlier indicate that few are reversed by
incubation in the presence of the enzyme and patient high density
lipoproteins.
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