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Identification of a platelet dense granule membrane protein that is
deficient in a patient with the Hermansky-Pudlak syndrome
JM Gerrard, D Lint, PJ Sims, T Wiedmer, RD Fugate, E McMillan, C Robertson and SJ Israels
Department of Paediatrics, University of Manitoba, Winnipeg, Canada.
Monoclonal antibodies were raised after injecting mice with isolated human
dense granules. Several of these monoclonals were found to recognize a
40-Kd dense granule membrane protein. Western blot and immunofluorescent
analysis confirmed the dense-granule specificity. After thrombin
activation, the protein was found in patches on the external platelet
membrane. By Western blot and slot blot analysis, the protein was found to
be markedly deficient in a patient with the Hermansky-Pudlak syndrome.
Studies of neutrophils and endothelial cells show the presence of
immunologically related granule-membrane protein(s). Western blots using
four anti-synaptophysin antibodies and three antibodies to the platelet
40-Kd protein suggest that the protein may share some homology with, but is
not identical to, the synaptosomal membrane protein synaptophysin.
Volume 77,
Issue 1,
pp. 101-112,
01/01/1991
Copyright © 1991 by The American Society of Hematology
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