Age-related deficiency of the synthesis of platelet activating factor by
leukocytes from Zellweger patients
A Sturk, MC Schaap, A Prins, JW ten Cate, LC Govaerts, RJ Wanders, HS Heymans and RB Schutgens
Ca2+-ionophore A23187-induced synthesis of the alkoxyether lipid platelet
activating factor (PAF) by leukocytes from Zellweger patients was
undetectable in two patients studied at 3 and 4 weeks of age, reduced in a
third patient studied at 2 months of age, and in the low normal range in
four patients studied between 4 months and 5 years of age. We have
previously reported that plasmalogen-type phosphatidylethanolamine (PE)
levels of erythrocytes are reduced in Zellweger patients up to 20 weeks of
age, but normal in older patients. These levels were reduced in the three
patients with abnormal PAF synthesis, and normal in the other four
patients. The results suggest a close relationship between the age of the
patients at sampling, and both the A23187-induced capacity of leukocytes to
synthesize PAF and the plasmalogen PE levels in their erythrocytes.
Volume 70,
Issue 2,
pp. 460-463,
08/01/1987
Copyright © 1987 by The American Society of Hematology
