ArticleHaematologica2009
Intrinsic impaired proplatelet formation and microtubule coil assembly of megakaryocytes in a mouse model of Bernard-Soulier syndrome.
Article in Haematologica, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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25 citing papers in PubMed, 76 citations in OpenAlex.
- Increased RhoA pathway activation downstream of αIIbβ3/SRC contributes to heterozygous Bernard Soulier syndrome.Haematologica · 2025Article
- Novel variants in GALE cause syndromic macrothrombocytopenia by disrupting glycosylation and thrombopoiesis.Blood · 2023Article
- Essential role of zyxin in platelet biogenesis and glycoprotein Ib-IX surface expression.Cell death & disease · 2021Article
- Megakaryopoiesis and Platelet Biology: Roles of Transcription Factors and Emerging Clinical Implications.International journal of molecular sciences · 2021Review
- miR-204-5p and Platelet Function Regulation: Insight into a Mechanism Mediated by CDC42 and GPIIbIIIa.Thrombosis and haemostasis · 2021Article
- Cytoskeletal-based mechanisms differently regulateHaematologica · 2021Article
- Structure-function of platelet glycoprotein Ib-IX.Journal of thrombosis and haemostasis : JTH · 2020Review
- New insights into cytoskeletal remodeling during platelet production.Journal of thrombosis and haemostasis : JTH · 2019Review
- Multiple concomitant mechanisms contribute to low platelet count in patients with immune thrombocytopenia.Scientific reports · 2019Article
- Microtubule plus-end tracking Adenopolyposis Coli negatively regulates proplatelet formation.Scientific reports · 2018Article
- Microtubule polyglutamylation and acetylation drive microtubule dynamics critical for platelet formation.BMC biology · 2018Article
- A Cdc42/RhoA regulatory circuit downstream of glycoprotein Ib guides transendothelial platelet biogenesis.Nature communications · 2017Article
- Evaluation of the immature platelet fraction contribute to the differential diagnosis of hereditary, immune and other acquired thrombocytopenias.Scientific reports · 2017Article
- LIM kinase/cofilin dysregulation promotes macrothrombocytopenia in severe von Willebrand disease-type 2B.JCI insight · 2016Article
- Hyaluronan Depolymerization by Megakaryocyte Hyaluronidase-2 Is Required for Thrombopoiesis.The American journal of pathology · 2016Article
- The contribution of mouse models to the understanding of constitutional thrombocytopenia.Haematologica · 2016Review
- Review
- Review
- Practical murine hematopathology: a comparative review and implications for research.Comparative medicine · 2015Review
- Stress-induced hematopoietic failure in the absence of immediate early response gene X-1 (IEX-1, IER3).Haematologica · 2014Article
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundGiant platelets and thrombocytopenia are invariable defects in the Bernard-Soulier syndrome caused by deficiency of the GPIb-V-IX complex, a receptor for von Willebrand factor supporting platelet adhesion to the damaged arterial wall. Various properties of this receptor may be considered potential determinants of the macrothrombocytopenia. DESIGN AND
methodsTo explore the underlying mechanisms of the disease, megakaryopoiesis was studied in a mouse model deficient in GPIbbeta. Megakaryocytes were initially characterized in situ in the bone marrow of adult mice, after which their capacity to differentiate into proplatelet-bearing cells was evaluated in cultured fetal liver cells.
resultsThe number of megakaryocyte progenitors, their differentiation and progressive maturation into distinct classes and their level of endoreplication were normal in GPIbbeta(-/-) bone marrow. However, the more mature cells exhibited ultrastructural anomalies with a thicker peripheral zone and a less well developed demarcation membrane system. GPIbbeta(-/-) megakaryocytes could be differentiated in culture from Lin(-) fetal liver cells in normal amounts but the proportion of cells able to extend proplatelets was decreased by 41%. Moreover, the GPIbbeta(-/-) cells extending proplatelets displayed an abnormal morphology characterized by fewer pseudopodial extensions with thicker shaft sections and an increased diameter of the terminal coiled elements. GPIbbeta(-/-) released platelets were larger but retained a typical discoid shape. Proplatelet formation was similarly affected in bone marrow explants from adult mice examined by videomicroscopy. The marginal microtubular ring contained twice as many tubulin fibers in GPIbbeta(-/-) proplatelet buds in cultured and circulating platelets.
conclusionsAltogether, these findings point to a role of the GPIb-V-IX complex intrinsic to megakaryocytes at the stage of proplatelet formation and suggest a functional link with the underlying microtubular cytoskeleton in platelet biogenesis.
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