Microtubule organization of unstimulated and stimulated adherent human
neutrophils in Chediak-Higashi syndrome
KB Pryzwansky, M Schliwa and LA Boxer
The numbers and length of centriole-associated microtubules of two patients
with Chediak-Higashi syndrome (CHS) were examined. Detergent- extracted
whole-mount preparations of adherent cells were studied by stereo
high-voltage electron microscopy. Under conditions of random migration,
neutrophils from both patients had a microtubule organization similar to
that of the control; microtubule numbers (28 +/- 3) and length (7.0 +/- 2.8
micron) were within normal range. When cells were treated with phorbol
myristate acetate (PMA), differences in the response of the two patients
were noted. Neutrophils from patient No. 2 and the control showed a
significant rise in numbers (38 +/- 5) and length (9.5 +/- 3.6 micron) of
microtubules. In contrast, neutrophils from patient No. 1 were unresponsive
to PMA treatment. Because vitamin C is used therapeutically in CHS patients
and has been shown to correct microtubule-related cell function,
neutrophils were exposed to ascorbic acid. A significant increase in
microtubule numbers (35 +/- 6) was observed in cells from the control and
patient No. 2 after ascorbate treatment; neutrophils from patient No. 1
showed no increase in microtubule numbers. While ascorbic acid did not
affect microtubule length in the control cells, it caused a significant
increase in microtubule length in neutrophils from both patients. Results
suggest that adherent CHS neutrophils contain centriole-associated
microtubules which are normal in number and length. However, differences
between patients are observed regarding neutrophil responsiveness to
stimuli which induce microtubule polymerization.
Volume 66,
Issue 6,
pp. 1398-1403,
12/01/1985
Copyright © 1985 by The American Society of Hematology
