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Hermansky‐pudlak platelets: Further studies on release reaction and protein phosphorylations
Author(s) -
Rendu Francine,
Maclouf Jacques,
Launay JeanMarie,
Boinot Catherine,
LevyToledano Sylviane,
Tanzer Joseph,
Caen Jacques
Publication year - 1987
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830250206
Subject(s) - platelet , arachidonic acid , chemistry , thrombin , ionophore , granule (geology) , lysosome , endocrinology , medicine , biochemistry , biology , enzyme , membrane , paleontology
Platelets from a patient with the Hermansky‐Pudlak syndrome were studied. These platelets had decreased amounts of serotonin and adenine nucleotides, and a decreased number of mepacrine‐labeled dense bodies. β‐Thromboglobulin and acid hydrolases contained in alpha‐granules and lysosomes respectively were present in normal amount. Platelets in platelet‐rich plasma did not respond to collagen, but arachidonic acid and ionophore A 23187 induced normal aggregation and normal thromboxane (TX) synthesis. Alpha‐granule release was found impaired and remained subnormal even with high doses of inducers. In response to thrombin aggregation, release and TX synthesis of isolated metrizamide gradient platelets were found at lower than normal levels. Phosphorylation of P20 and P43 proteins was normal. Only a combination of ADP plus thrombin could restore a normal aggregation, with normal alpha‐granule and lysosome release and normal TX synthesis. These results indicated that in the absence of dense bodies: 1) the release of other granules is impaired; 2) the TX synthesis is delayed except when induced by arachidonic acid and A 23187 ionophore; 3) the absence of dense bodies could be compensated for by the addition of ADP which restores the impaired release reaction and TX formation; and 4) P20 and P43 polypeptides were phosphorylated as rapidly as those in normal platelets.