Journal of Lipid Research, Vol 24, 1605-1611, Copyright © 1983 by Lipid Research, Inc.
Cholesterol turnover and metabolism in two patients with abetalipoproteinemia
DS Goodman, RJ Deckelbaum, RH Palmer, RB Dell, R Ramakrishnan, G Delpre, Y Beigel and M Cooper
Total body turnover of cholesterol was studied in two patients with
abetalipoproteinemia, a 32-year-old man and a 31-year-old woman. The
patients received [14C]cholesterol intravenously, and the resulting
specific activity-time curves (for 40 and 30 weeks, respectively) were
fitted with a three-pool model. Parameters were compared with those from
studies of cholesterol turnover in 82 normal and hyperlipidemic subjects. A
three-pool model gave the best fit for the abetalipoproteinemic patients,
as well as for the 82 previously studied subjects, suggesting general
applicability of this model. Cholesterol production rates in the two
abetalipoproteinemic subjects (0.82 and 0.89 g/day) were close to values
predicted for persons of their body weight. Thus, total body turnover rate
of cholesterol was quite normal in abetalipoproteinemia, confirming
previous reports. Very low values (9.2 and 8.4 g) were found for M1, the
size of the rapidly exchanging compartment pool 1, in the two
abetalipoproteinemic subjects. These values were well below the values
predicted (from the comparison study population) for normal persons of this
size with low plasma cholesterol levels. For one patient, total body
exchangeable cholesterol was very low, although not significantly below the
predicted values for a person of his size. In the second patient, the
observed estimate for total body exchangeable cholesterol was well within
the range of values predicted for persons of her size with low to extremely
low cholesterol levels.(ABSTRACT TRUNCATED AT 250 WORDS)
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