HOMEPRODUCTSSERVICESCOMPANYCONTACTFAQResearchDictionaryPharmaMobileSign Up FREE or Login

Studies on a new variant of the Hermansky-Pudlak syndrome: qualitative, ultrastructural, and functional abnormalities of the platelet-dense bodies associated with a phospholipase A defect.

Abstract
The structure and functions of platelets from a patient in whom albinism and hemorrhagic diathesis were associated have been investigated. Electron microscope studies showed a large reduction in the number of dense bodies and this was confirmed by an examination of fluorescent platelets loaded with mepacrine. The rare dense bodies were much bigger than normally observed; their density was diminished and was localized in a peripheral ring. Other platelet constituents were found to be normal. Platelet peroxidase activity was normal in the canaliculi of the dense tubular system; catalase-positive granules were also present. Serotonin uptake by the patient's platelets was much decreased and reserpine, a potent inhibitor of serotonin accumulation by normal human platelets, did not further decrease this incorporation. The uptake of free 14 C-arachidonic acid by the platelets was greatly diminished, as was its thrombin-induced liberation from phosphatidyl-choline and phosphatidyl inositol. Moreover, platelet phospholipase A1 activity was much reduced and phospholipase A2 activity was undetectable.
AuthorsF Rendu, J Breton-Gorius, G Trugnan, H C Malaspina, J M Andrieu, G Bereziat, M Lebret, J P Caen
JournalAmerican journal of hematology (Am J Hematol) Vol. 4 Issue 4 Pg. 387-99 ( 1978) ISSN: 0361-8609 [Print] UNITED STATES
PMID717398 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Arachidonic Acids
  • Serotonin Antagonists
  • Serotonin
  • Reserpine
  • Phospholipases
Topics
  • Adult
  • Arachidonic Acids (metabolism)
  • Blood Platelet Disorders (blood)
  • Blood Platelets (ultrastructure)
  • Female
  • Genetic Variation
  • Humans
  • Megakaryocytes (ultrastructure)
  • Phospholipases (deficiency)
  • Reserpine (pharmacology)
  • Serotonin (metabolism)
  • Serotonin Antagonists
  • Syndrome

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!


Choose Username:
Email:
Password:
Verify Password: