Article
An incomplete trafficking defect to the cell-surface leads to paradoxical thrombocytosis for human and murine MPL P106L.
Blood - 29 Dec 2016
Favale Fabrizia, Messaoudi Kahia, Varghese Leila N, Boukour Siham, Pecquet Christian, Gryshkova Vitalina, Defour Jean Philippe, Albu Roxana-Irina, Bluteau Olivier, Ballerini Paola, Leverger Guy, Plo Isabelle, Debili Najet, Raslova Hana, Favier Remi, Constantinescu Stefan N, Vainchenker William
Abstract excerpt
The mechanisms behind the hereditary thrombocytosis induced by the thrombopoietin (THPO) receptor MPL P106L mutant remain unknown. A complete trafficking defect to the cell surface has been reported, suggesting either weak constitutive activity or nonconventional THPO-dependent mechanisms. Here, we report that the thrombocytosis phenotype induced by MPL P106L belongs to the paradoxical group, where low MPL levels...
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