Haematologica (Apr 2020)

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

DOI
https://doi.org/10.3324/haematol.2018.214460
Journal volume & issue
Vol. 105, no. 4

Abstract

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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