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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Novak, E. K. Articles by Swank, R. T. Search for Related Content PubMed PubMed Citation Articles by Novak, E. K. Articles by Swank, R. T. Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Correction of symptoms of platelet storage pool deficiency in animal models for Chediak-Higashi syndrome and Hermansky-Pudlak syndrome EK Novak, MP McGarry and RT Swank Two human diseases of platelet storage pool deficiency (SPD), Hermansky- Pudlak syndrome and Chediak-Higashi syndrome, are recessively inherited disorders characterized by hypopigmentation, prolonged bleeding, and normal platelet counts accompanied by a reduction in dense granule number. We have recently described seven independent recessive mutations in the mouse regulated by separate genes which are likely animal models for human SPD. Reciprocal bone marrow transplants were carried out between normal C57BL/6J mice and two of these mutants, beige and pallid, in order to test whether the platelet defects are due to a defect in platelet progenitor cells or to humoral factors. Normal and congenic mutant mice were transplanted with marrow after 950 rad whole body radiation. The long bleeding times and low serotonin concentrations of the two mutants were converted to normal values after transplantation with normal marrow. Likewise, normal mice displayed symptoms of SPD when transplanted with mutant marrow. These studies demonstrate that with each of the two mutations, platelet SPD results from a defect in bone marrow precursor cells. Also, the studies suggest that in severe cases, platelet SPD may be successfully treated by bone marrow transplantation. Volume 66, Issue 5, pp. 1196-1201, 11/01/1985 Copyright © 1985 by The American Society of Hematology CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: D. A. Wong, Y. Kita, N. Uozumi, and T. Shimizu Discrete Role for Cytosolic Phospholipase A2{alpha} in Platelets: Studies Using Single and Double Mutant Mice of Cytosolic and Group IIA Secretory Phospholipase A2 J. Exp. Med., August 5, 2002; 196(3): 349 - 357. [Abstract] [Full Text] [PDF]

	Copyright © 1985 by American Society of Hematology         Online ISSN: 1528-0020