Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets
Sergey I. Obydennyi,
Elena O. Artemenko,
Anastasia N. Sveshnikova,
Anastasia A. Ignatova,
Tatiana V. Varlamova,
Stepan Gambaryan,
Galina Y. Lomakina,
Natalia N. Ugarova,
Igor I. Kireev,
Fazoil I. Ataullakhanov,
Galina A. Novichkova,
Aleksey A. Maschan,
Anna Shcherbina,
Mikhail Panteleev
Affiliations
Sergey I. Obydennyi
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow;Center for Theoretical Problems of Physicochemical Pharmacology, Moscow
Elena O. Artemenko
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow;Center for Theoretical Problems of Physicochemical Pharmacology, Moscow
Anastasia N. Sveshnikova
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow;Center for Theoretical Problems of Physicochemical Pharmacology, Moscow;Faculty of Physics, Lomonosov Moscow State University, Moscow;I.M. Sechenov First Moscow State Medical University, Moscow
Anastasia A. Ignatova
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow;Center for Theoretical Problems of Physicochemical Pharmacology, Moscow
Tatiana V. Varlamova
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow
Stepan Gambaryan
Sechenov Institute of Evolutionary Physiology and Biochemistry, Russian Academy of Sciences, St Petersburg
Galina Y. Lomakina
Department of Chemistry, Lomonosov Moscow State University, Moscow;Bauman Moscow State Technical University, Moscow
Natalia N. Ugarova
Department of Chemistry, Lomonosov Moscow State University, Moscow
Igor I. Kireev
Belozersky Institute of Physico-Chemical Biology, Lomonosov Moscow State University, Moscow
Fazoil I. Ataullakhanov
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow;Center for Theoretical Problems of Physicochemical Pharmacology, Moscow;Faculty of Physics, Lomonosov Moscow State University, Moscow;Faculty of Biological and Medical Physics, Moscow Institute of Physics and Technology, Dolgoprudny, Russia
Galina A. Novichkova
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow
Aleksey A. Maschan
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow
Anna Shcherbina
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow
Mikhail Panteleev
National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow;Center for Theoretical Problems of Physicochemical Pharmacology, Moscow;Faculty of Physics, Lomonosov Moscow State University, Moscow;Faculty of Biological and Medical Physics, Moscow Institute of Physics and Technology, Dolgoprudny, Russia
Wiskott-Aldrich syndrome (WAS) is associated with thrombocytopenia of unclear origin. We investigated real-time cytosolic calcium dynamics, mitochondrial membrane potential and phoszphatidylserine (PS) exposure in single fibrinogen-bound platelets using confocal microscopy. The WAS platelets had higher resting calcium levels, more frequent spikes, and their mitochondria more frequently lost membrane potential followed by PS exposure (in 22.9% of platelets vs. 3.9% in controls; P