{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T13:58:55Z","timestamp":1758635935122},"reference-count":35,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":6980,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1987,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Platelets from a patient with the Hermansky\u2010Pudlak syndrome were studied. These platelets had decreased amounts of serotonin and adenine nucleotides, and a decreased number of mepacrine\u2010labeled dense bodies. \u03b2\u2010Thromboglobulin and acid hydrolases contained in alpha\u2010granules and lysosomes respectively were present in normal amount. Platelets in platelet\u2010rich plasma did not respond to collagen, but arachidonic acid and ionophore A 23187 induced normal aggregation and normal thromboxane (TX) synthesis. Alpha\u2010granule 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\u2010granule 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.<\/jats:p>","DOI":"10.1002\/ajh.2830250206","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T11:09:32Z","timestamp":1172056172000},"page":"165-174","source":"Crossref","is-referenced-by-count":19,"title":["Hermansky\u2010pudlak platelets: Further studies on release reaction and protein phosphorylations"],"prefix":"10.1002","volume":"25","author":[{"given":"Francine","family":"Rendu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacques","family":"Maclouf","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean\u2010Marie","family":"Launay","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catherine","family":"Boinot","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylviane","family":"Levy\u2010Toledano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph","family":"Tanzer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacques","family":"Caen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"163","article-title":"Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: Report of two cases with histochemical studies","volume":"4","author":"Hermansky F","year":"1959","journal-title":"Blood"},{"key":"e_1_2_1_3_2","first-page":"590","article-title":"Ultrastructural features of abnormal blood platelets","volume":"83","author":"White JG","year":"1976","journal-title":"Am J Pathol"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1972.tb03483.x"},{"key":"e_1_2_1_5_2","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1182\/blood.V39.2.197.197","article-title":"Further evidence for a deficient storage pool of adenine nucleotides in platelets from some patients with thrombocytopenia \u201cstorage pool disease","volume":"39","author":"Holmsen H","year":"1972","journal-title":"Blood"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI107778"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1055\/s-0038-1666908","article-title":"Relationship between mepacrine\u2010labelled dense body number, platelet capacity to accumulate [14C] 5\u2010HT and platelet density in the Bernard\u2010Soulier and Hermansky\u2010Pudlak syndrome","volume":"42","author":"Rendu F","year":"1979","journal-title":"Thromb Haemost"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830040410"},{"key":"e_1_2_1_9_2","first-page":"831","article-title":"Gray\u2010platelet syndrome: \u03b1\u2010granule deficiency","volume":"98","author":"Levy\u2010Toledano S","year":"1981","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(83)90553-3"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-2836(71)90148-3"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(72)90323-5"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(80)90296-1"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1021\/ac00284a003"},{"key":"e_1_2_1_15_2","first-page":"277","volume-title":"Platelet Cellular Response Mechanisms and Their Biological Significance","author":"Da Prada M","year":"1980"},{"key":"e_1_2_1_16_2","first-page":"605","article-title":"Control of pineal N\u2010acetyl\u2010serotonin by a beta\u2010adrenergic receptor","volume":"9","author":"Browstein M","year":"1973","journal-title":"Mol Pharmacol"},{"key":"e_1_2_1_17_2","unstructured":"BarreN:Etude sur les hydroxy et les m\u00e9thoxy\u2010indoles plaquettaires: Mise en \u00e9vidence dune voie de biosynth\u00e8se de la m\u00e9latonine. 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