Biochemical studies of two patients with the gray platelet syndrome. Selective deficiency of platelet alpha granules.

摘要: The biochemistry of platelets from two unrelated patients with the gray platelet syndrome, a deficiency alpha-granules, has been evaluated. Ultrastructural studies their revealed number alpha-granules to be less than 15% normal, whereas dense bodies was within normal limits. Platelets both had severe deficiencies factor 4 and beta-thromboglobulin (less 10% normal). Sodium dodecyl sulfate-polyacrylamide gel electrophoresis showed marked thrombin-sensitive protein in patients. Analysis platelet-derived growth one patient it also markedly reduced. Levels lysosomal enzymes, adenine nucleotides, serotonin, catalase, conversion arachidonic acid by lipoxygenase cyclo-oxygenase were results provide important evidence define contents differentiate these granules, bodies, peroxisomes. Functional ADP, thrombin, collagen aggregation. suggest that or make contribution