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Inherited abnormalities in platelet organelles and platelet formation and
associated altered expression of low molecular weight guanosine
triphosphate-binding proteins in the mouse pigment mutant gunmetal
RT Swank, SY Jiang, M Reddington, J Conway, D Stephenson, MP McGarry and EK Novak
Dept. of Molecular and Cellular Biology, Roswell Park Cancer Institute,
Buffalo, NY 14263.
Gunmetal (gm/gm) is a recessively inherited mouse pigment dilution mutant
that has high mortality and poor reproductive rates. In these studies,
several hematologic defects were found associated with the mutation,
including prolonged bleeding times, together with thrombocytopenia and
increased platelet size. A unique feature is the presence of simultaneous
abnormalities in two platelet organelles, dense granules and
alpha-granules. The dense granule component serotonin is present at about
half the normal concentration, as are visible dense granules. Three
alpha-granule components (fibrinogen, platelet factor 4, and von Willebrand
factor) are also significantly reduced. Thus, in several respects the
gunmetal mutant resembles the human gray platelet syndrome. A novel
abnormality in expression of low molecular weight guanosine triphosphate
(GTP)-binding proteins occurs in platelets of gunmetal. In Western blot
assays, two additional GTP- binding proteins of 28.5 and 25 Kd were
detected. The abnormal expression of GTP-binding proteins is, like the
hematologic defects, genetically recessive and is tissue specific. Liver,
kidney, brain, spleen, macrophages, and neutrophils have normal GTP-binding
protein expression. The additional GTP-binding proteins are soluble. The
data indicate that platelet formation and platelet organelle biogenesis are
under common genetic control and that abnormal regulation of GTP- binding
proteins may affect one or both processes.
Volume 81,
Issue 10,
pp. 2626-2635,
05/15/1993
Copyright © 1993 by The American Society of Hematology
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