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Immature dense granules in platelets from mice with platelet storage pool
disease
M Reddington, EK Novak, E Hurley, C Medda, MP McGarry and RT Swank
Mepacrine uptake into platelets and bone marrow megakaryocytes was analyzed
to further characterize the dense granule defects in a group of seven mouse
pigment mutants that have characteristics of platelet storage pool disease
(SPD). In contrast to our previous studies using electron microscopy, this
method revealed that all mutants had normal numbers of dense granules.
However, total mepacrine uptake in all mutant platelets was significantly
diminished to less than 50% of normal uptake. Also, the flashing phenomenon
observed when normal dense granules are irradiated with ultraviolet light
was either greatly diminished or absent when platelets of individual
mutants were similarly irradiated. Therefore the principal defect in the
mutant platelets is an inability to accumulate dense granule contents
rather than an absence of the granules. Mepacrine uptake into
megakaryocytes was indistinguishable in normal and mutant mice. This
indicates the mutant dense granule defects appear either very late in
megakaryocyte development or early in platelet formation in correlation
with development of the mature dense granule. By standard transmission
electron microscopy we have not been able to detect gross structural or
subcellular abnormalities in either platelets or megakaryocytes of mutant
mice. It appears all seven mutants produce immature or functionally
abnormal dense granules.
Volume 69,
Issue 5,
pp. 1300-1306,
05/01/1987
Copyright © 1987 by The American Society of Hematology
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