Hermansky-Pudlak综合征的生化研究。
Biochemical studies in Hermansky-Pudlak syndrome.
作者信息
Gerritsen S M, Akkerman J W, Staal G, Roelofsen B, Koster J F, Sixma J J
出版信息
Scand J Haematol. 1979 Aug;23(2):161-8. doi: 10.1111/j.1600-0609.1979.tb02687.x.
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 incubation with diamide (a specific agent for GSH oxidation). Phospholipid and fatty acid composition of HPS platelets was normal. The amount of peroxides found in platelet membranes was not elevated. A subnormal aggregation with arachidonic acid could be obtained in PRP using a high concentration of arachidonic acid (2 mM), but normal malondialdehyde levels were measured, suggesting a normal prostaglandin synthesis in HPS platelets. Glutathion peroxidase and p-phenylenediamide-mediated peroxidase (PPD-peroxidase) were normal in leucocytes of 1 HPS patient. Lysosomal enzymes as far as investigated were normal.
为了找出赫尔曼斯基-普德拉克综合征（HPS）的基本缺陷，我们对血小板和白细胞进行了生化研究。血小板的谷胱甘肽水平正常，在用二酰胺（一种用于谷胱甘肽氧化的特异性试剂）孵育后，谷胱甘肽的再生情况与对照组相似。HPS血小板的磷脂和脂肪酸组成正常。在血小板膜中发现的过氧化物量没有升高。使用高浓度的花生四烯酸（2 mM）在富血小板血浆（PRP）中可获得与花生四烯酸的亚正常聚集，但测得的丙二醛水平正常，这表明HPS血小板中的前列腺素合成正常。1例HPS患者白细胞中的谷胱甘肽过氧化物酶和对苯二胺介导的过氧化物酶（PPD-过氧化物酶）正常。就所研究的溶酶体酶而言，其是正常的。
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