Article
The abnormal proplatelet formation in MYH9-related macrothrombocytopenia results from an increased actomyosin contractility and is rescued by myosin IIA inhibition.
Journal of thrombosis and haemostasis : JTH - 1 Dec 2013
Chen Y, Boukour S, Milloud R, Favier R, Saposnik B, Schlegel N, Nurden A, Raslova H, Vainchenker W, Balland M, Nurden P, Debili N
Abstract excerpt
BACKGROUND: Mutations in the MYH9 gene cause autosomal dominant MYH9-related diseases (MYH9-RD) that associate macrothrombocytopenia with various other clinical conditions. The mechanisms giving rise to giant platelets remain poorly understood. OBJECTIVES/PATIENTS: To study the proplatelet formation (PPF) derived from megakaryocytes (MKs) generated in vitro from 11 patients with MYH9-RD with different mutations,...
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