{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T12:01:13Z","timestamp":1780056073217,"version":"3.54.0"},"reference-count":47,"publisher":"American Society of Hematology","issue":"3","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2002,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Phospholipase C (PLC)\u2013\u03b22 plays a major role in platelet activation. Previous studies have described a unique patient with impaired receptor-mediated platelet aggregation, secretion, calcium mobilization, and phospholipase C (PLC) activation associated with a selective decrease in platelet PLC-\u03b22 isozyme. To identify the mechanisms leading to the defect, platelet RNA from the patient and healthy subjects was subjected to reverse transcription\u2013polymerase chain reaction (RT-PCR) and the products sequenced. The PLC-\u03b22 cDNA sequence in the patient showed no abnormalities. Platelet PLC-\u03b22 and \u03b2-actin (internal control) mRNA levels were assessed by RT-PCR; the ratio of PLC-\u03b22 to \u03b2-actin mRNA levels was 0.80 to 0.95 in 4 healthy subjects and 0.28 in the patient. PLC-\u03b22 mRNA levels were similarly reduced compared with GPIIb and G\u03b1q mRNA levels. PLC-\u03b32 and platelet factor 4 mRNA levels were normal. Calcium mobilization was studied in neutrophils upon activation with formyl-Met-Leu-Phe (fMLP), adenosine diphosphate (ADP), platelet-activating factor (PAF), interleukin-8 (IL-8), C5a, and leukotriene B4 (LTB4), and it was normal. Neutrophil elastase secretion upon activation with fMLP, ADP, PAF, IL-8, C5a, and LTB4 was normal, as were neutrophil PLC-\u03b22 mRNA and PLC-\u03b22 on immunoblotting. Thus, responses to activation, PLC-\u03b22 protein, and PLC-\u03b22 mRNA are decreased in patient platelets but not in neutrophils, providing evidence for a hitherto undescribed lineage (platelet)\u2013specific defect in PLC-\u03b22 gene expression. These studies provide a physiologically relevant model to delineate regulation of PLC-\u03b22 gene and its tissue-specific expression.<\/jats:p>","DOI":"10.1182\/blood.v99.3.905","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T12:46:40Z","timestamp":1027774000000},"page":"905-911","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":26,"title":["Lineage-specific defect in gene expression in human platelet phospholipase C-\u03b22 deficiency"],"prefix":"10.1182","volume":"99","author":[{"given":"Guang Fen","family":"Mao","sequence":"first","affiliation":[{"name":"From the Sol Sherry Thrombosis Research Center, and the Departments of Medicine and Physiology, Temple University School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vijender R.","family":"Vaidyula","sequence":"additional","affiliation":[{"name":"From the Sol Sherry Thrombosis Research Center, and the Departments of Medicine and Physiology, Temple University School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satya P.","family":"Kunapuli","sequence":"additional","affiliation":[{"name":"From the Sol Sherry Thrombosis Research Center, and the Departments of Medicine and Physiology, Temple University School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. 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