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pro vyhledávání: '"S. M. Gerritsen"'
Publikováno v:
Scandinavian Journal of Haematology. 23:161-168
In order to find the basic defect in the Hermansky-Pudlak Syndrome (HPS), biochemical studies of platelets and leucocytes were undertaken. Glutathione levels of platelets were normal and regeneration of GSH similar to controls occurred after incubati
Autor:
B. Nijmeijer, Carl J. Witkop, J. W. N. Akkerman, James G. White, S. M. Gerritsen, J. J. Sixma
Publikováno v:
Scandinavian Journal of Haematology. 18:249-256
A Dutch kindred with the Hermansky-Pudlak syndrome (HPS) is described. We show for the first time evidence of a lowered platelet 5-hydroxytryptamine content in obligate heterozygotes. Platelet ATP and ADP levels and ATP/ADP ratio were normal in these
Publikováno v:
Scandinavian journal of haematology. 18(3)
A Dutch kindred with the Hermansky-Pudlak syndrome (HPS) is described. We show for the first time evidence of a lowered platelet 5-hydroxytryptamine content in obligate heterozygotes. Platelet ATP and ADP levels and ATP/ADP ratio were normal in these
Publikováno v:
VIth International Congress on Thrombosis and Haemostasis.
Four patients with Hermansky-Pudlak Syndroora (HPS)— storage pool deficiency, albinism and ceroid containing bone marrow macrophages — from one family, one unrelated hps patient and one patient with storage pool deficiency (SPD) were alternately
Publikováno v:
The American journal of pathology. 70(3)
The present investigation has explored the fine structure of the lipid inclusions which fill the bone marrow macrophages of patients with the Hermansky-Pudlak syndrome. Red blood cells are the major substrate of the reticular macrophages, and incompl