Open Access
Haematologica, volume 105, issue 4, pages 1095-1106
Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets
Publication type: Journal Article
Publication date: 2019-07-05
Journal:
Haematologica
Quartile SCImago
Q1
Quartile WOS
Q1
Impact factor: 10.1
ISSN: 03906078, 15928721
PubMed ID:
31278208
Hematology
Abstract
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<0.001) after the collapse of the last mitochondria. This phenomenon was inhibited by the mitochondrial permeability transition pore inhibitor cyclosporine A, as well by xestospongin C and lack of extracellular calcium. Thapsigargin by itself caused accelerated cell death in the WAS platelets. The number of mitochondria was predictive of PS exposure: 33% of platelets from WAS patients with fewer than five mitochondria exposed PS, while only 12% did among those that had five or more mitochondria. Interestingly, healthy donor platelets with fewer mitochondria also more readily became procoagulant upon PAR1/PAR4 stimulation. Collapse of single mitochondria led to greater cytosolic calcium increase in WAS platelets if they had one to three mitochondria compared with platelets containing higher numbers. A computer systems biology model of platelet calcium homeostasis showed that smaller platelets with fewer mitochondria could have impaired calcium homeostasis because of higher surface-to-volume ratio and greater metabolic load, respectively. There was a correlation (C=0.81, P<0.02) between the mean platelet size and platelet count in the WAS patients. We conclude that WAS platelets readily expose PS via a mitochondria-dependent necrotic mechanism caused by their smaller size, which could contribute to the development of thrombocytopenia.
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- We do not take into account publications that without a DOI.
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- Statistics recalculated weekly.
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Artemenko E. O. et al. Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets // Haematologica. 2019. Vol. 105. No. 4. pp. 1095-1106.
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Artemenko E. O., Sveshnikova A. N., Ignatova A., Varlamova T. V., Gambaryan S., Lomakina M. E., Ugarova N. N., Kireev I. I., Novichkova A. A., Maschan A. A., Panteleev M. A., Ataullakhanov F. I., Shcherbina A., Obydennyi S. I. Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets // Haematologica. 2019. Vol. 105. No. 4. pp. 1095-1106.
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TY - JOUR
DO - 10.3324/haematol.2018.214460
UR - https://doi.org/10.3324%2Fhaematol.2018.214460
TI - Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets
T2 - Haematologica
AU - Artemenko, Elena O.
AU - Sveshnikova, Anastasia N.
AU - Ignatova, Anastasia
AU - Varlamova, Tatiana V
AU - Gambaryan, Stepan
AU - Lomakina, M E
AU - Ugarova, Natalia N
AU - Kireev, Igor I
AU - Novichkova, A. A.
AU - Maschan, Aleksey A
AU - Panteleev, M. A.
AU - Ataullakhanov, Fazoil I.
AU - Shcherbina, Anna
AU - Obydennyi, Sergey I
PY - 2019
DA - 2019/07/05 00:00:00
PB - Ferrata Storti Foundation
SP - 1095-1106
IS - 4
VL - 105
PMID - 31278208
SN - 0390-6078
SN - 1592-8721
ER -
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@article{2019_Artemenko,
author = {Elena O. Artemenko and Anastasia N. Sveshnikova and Anastasia Ignatova and Tatiana V Varlamova and Stepan Gambaryan and M E Lomakina and Natalia N Ugarova and Igor I Kireev and A. A. Novichkova and Aleksey A Maschan and M. A. Panteleev and Fazoil I. Ataullakhanov and Anna Shcherbina and Sergey I Obydennyi},
title = {Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets},
journal = {Haematologica},
year = {2019},
volume = {105},
publisher = {Ferrata Storti Foundation},
month = {jul},
url = {https://doi.org/10.3324%2Fhaematol.2018.214460},
number = {4},
pages = {1095--1106},
doi = {10.3324/haematol.2018.214460}
}
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MLA
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Artemenko, Elena O., et al. “Mechanisms of increased mitochondria-dependent necrosis in Wiskott-Aldrich syndrome platelets.” Haematologica, vol. 105, no. 4, Jul. 2019, pp. 1095-1106. https://doi.org/10.3324%2Fhaematol.2018.214460.