Study of Bernard–Soulier Syndrome Megakaryocytes and Platelets Using Patient-Derived Induced Pluripotent Stem Cells
| Autor: | Nipan Israsena, Ponlapat Rojnuckarin, Supang Maneesri Le Grand, Praewphan Ingrungruanglert, Darintr Sosothikul, Ponthip Mekchay, Wilawan Ji-au, Kanya Suphapeetiporn |
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| Rok vydání: | 2019 |
| Předmět: |
Blood Platelets
0301 basic medicine Cellular differentiation Induced Pluripotent Stem Cells Cell- and Tissue-Based Therapy 030204 cardiovascular system & hematology Immunofluorescence Bernard–Soulier syndrome Cell Line 03 medical and health sciences 0302 clinical medicine medicine Humans Cellular Reprogramming Techniques Platelet Induced pluripotent stem cell Blood Coagulation Cell Shape medicine.diagnostic_test Chemistry Cell Membrane Lentivirus Bernard-Soulier Syndrome Cell Differentiation Genetic Therapy Hematology medicine.disease Cell biology Microscopy Electron GP1BA 030104 developmental biology Platelet Glycoprotein GPIb-IX Complex Cell culture Female sense organs Megakaryocytes |
| Zdroj: | Thrombosis and Haemostasis. 119:1461-1470 |
| ISSN: | 2567-689X 0340-6245 |
| DOI: | 10.1055/s-0039-1693409 |
| Popis: | Bernard–Soulier syndrome (BSS) is a hereditary macrothrombocytopenia caused by defects in the glycoprotein (GP) Ib-IX-V complex. The mechanism of giant platelet formation remains undefined. Currently, megakaryocytes (MKs) can be generated from induced pluripotent stem cells (iPSCs) to study platelet production under pharmacological or genetic manipulations. Here, we generated iPSC lines from two BSS patients with mutations in different genes (GP1BA and GP1BB: termed BSS-A and BSS-B, respectively). The iPSC-derived MKs and platelets were examined under electron microscopy and stained by immunofluorescence to observe proplatelet formation and measure platelet diameters which were defined by circumferential tubulin. BSS-iPSCs produced abnormal proplatelets with thick shafts and tips. In addition, compared with the normal iPSCs, the diameters were larger in platelets derived from BSS-A and BSS-B with the means ± standard deviations of 4.34 ± 0.043 and 3.88 ± 0.045 µm, respectively (wild-type iPSCs 2.61 ± 0.025 µm, p |
| Databáze: | OpenAIRE |
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