A B S T R A C T The platelets from three patients withBernard-Soulier syndrome have been analyzed by surface-labeling coupled with two-dimensional gel electrophoresis and compared with normals. As well as the previously described absence or deficiency in glycoprotein (GP) Ib(a) it could be shown that GP Ib,3 and an additional low molecular weight glycoprotein GP75.8-65 were not detectable using carbohydrate-labeling methods or deficient to the same extent as the GPIba subunit. In addition, the thrombin cleavable glycoprotein could not be detected using carbohydrate-labeling methods in two patients and was deficient in a third. This finding was confirmed in a fourth patient by one-dimensional gel electrophoresis. Thus, the changes in the membrane of Bernard-Soulier platelets are more complex than previously thought.
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