ArticleJCI insight2016
LIM kinase/cofilin dysregulation promotes macrothrombocytopenia in severe von Willebrand disease-type 2B.
Article in JCI insight, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 29 citations in OpenAlex.
- CryoET reveals actin filaments within platelet microtubules.Nature communications · 2024Article
- Efficacy of platelet-inspired hemostatic nanoparticles on bleeding in von Willebrand disease murine models.Blood · 2023Article
- The role of CREG1 in megakaryocyte maturation and thrombocytopoiesis.International journal of biological sciences · 2023Article
- The impact of aberrant von Willebrand factor-GPIbα interaction on megakaryopoiesis and platelets in humanized type 2B von Willebrand disease model mouse.Haematologica · 2022Article
- Platelet dysfunction in platelet-type von Willebrand disease due to the constitutive triggering of the Lyn-PECAM1 inhibitory pathway.Haematologica · 2022Article
- Don't you forget about me(gakaryocytes).Blood · 2022Article
- The Role of LIM Kinases during Development: A Lens to Get a Glimpse of Their Implication in Pathologies.Cells · 2022Review
- A thrombopoietin receptor agonist to rescue an unusual platelet transfusion-induced reaction in a p.V1316M-associated von Willebrand disease type 2B patient.Therapeutic advances in hematology · 2022Article
- Endoglin Is an Endothelial Housekeeper against Inflammation: Insight in ECFC-Related Permeability through LIMK/Cofilin Pathway.International journal of molecular sciences · 2021Article
- Structure-function of platelet glycoprotein Ib-IX.Journal of thrombosis and haemostasis : JTH · 2020Review
- Actin/microtubule crosstalk during platelet biogenesis in mice is critically regulated by Twinfilin1 and Cofilin1.Blood advances · 2020Article
- Relevance of platelet desialylation and thrombocytopenia in type 2B von Willebrand disease: preclinical and clinical evidence.Haematologica · 2019Article
- Hyperactive GPIb-von Willebrand factor interaction as cause of thrombocytopenia: altered platelet formationHaematologica · 2019Article
- Mechanisms of thrombocytopenia in platelet-type von Willebrand disease.Haematologica · 2019Article
- Advances in Clinical and Basic Science of Coagulation: Illustrated abstracts of the 9th Chapel Hill Symposium on Hemostasis.Research and practice in thrombosis and haemostasis · 2018Review
- Nbeal2 interacts with Dock7, Sec16a, and Vac14.Blood · 2018Article
- LIM kinases: cofilin and beyond.Oncotarget · 2017Review
- A Cdc42/RhoA regulatory circuit downstream of glycoprotein Ib guides transendothelial platelet biogenesis.Nature communications · 2017Article
- Role of Rho-GTPases in megakaryopoiesis.Small GTPasesReview
Corrections and comments
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Authors and funding
18 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
von Willebrand disease type 2B (VWD-type 2B) is characterized by gain-of-function mutations of von Willebrand factor (vWF) that enhance its binding to platelet glycoprotein Ibα and alter the protein's multimeric structure. Patients with VWD-type 2B display variable extents of bleeding associated with macrothrombocytopenia and sometimes with thrombopathy. Here, we addressed the molecular mechanism underlying the severe macrothrombocytopenia both in a knockin murine model for VWD-type 2B by introducing the p.V1316M mutation in the murine
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.