ArticleRare diseases (Austin, Tex.)2013
The Nbeal2(-/-) mouse as a model for the gray platelet syndrome.
Article in Rare diseases (Austin, Tex.), 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 27 citations in OpenAlex.
- Procoagulant platelets: linking coagulation and thromboinflammation in cardiovascular disease.Nature reviews. Cardiology · 2026Review
- Platelet α-granule cargo packaging and endocytosis are important for normal mouse skin wound healing.Blood advances · 2026Article
- Nbeal2 Inactivation Triggers Abl1 Stabilisation and Dysregulated Subcellular Localisation of the Multi-Drug-Resistant Protein MDR1 (ABCB1) in Mast Cells.Immunology · 2026Article
- Serglycin controls megakaryocyte retention of platelet factor 4 and influences megakaryocyte fate in bone marrow.Blood advances · 2025Article
- NBEAL2 deficiency in humans leads to low CTLA-4 expression in activated conventional T cells.Nature communications · 2023Article
- Platelet α-granules are required for occlusive high-shear-rate thrombosis.Blood advances · 2020Article
- Occlusive thrombosis in arteries.APL bioengineering · 2019Review
- The road to lysosome-related organelles: Insights from Hermansky-Pudlak syndrome and other rare diseases.Traffic (Copenhagen, Denmark) · 2019Review
- Platelets as Modulators of Cerebral Ischemia/Reperfusion Injury.Frontiers in immunology · 2019Review
- The contribution of mouse models to the understanding of constitutional thrombocytopenia.Haematologica · 2016Review
- Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The gray platelet syndrome (GPS) is a rare, autosomal-recessive platelet disorder characterized by thrombocytopenia, large platelets lacking α-granules, and variable bleeding. GPS has been linked to mutations in the neurobeachin-like 2 gene (NBEAL2). We have recently characterized Nbeal2-deficient mice and shown that the absence of Nbeal2 results in defective protein sorting in megakaryocytes (MKs) and impaired α-granule biogenesis, a finding also seen for human MKs. In the mice, the lack of α-granules results in impaired aggregation, defective platelet adhesion to collagen under flow and reduced pro-coagulant activity; findings that translate into defective hemostasis and thrombosis in vivo indicating that α-granule secretion is critical for platelet plug stability. Furthermore, we revealed a role of α-granule proteins in ischemic stroke and wound healing. Thus, Nbeal2-deficient mice recapitulate the hallmarks of human GPS without showing its phenotypic heterogeneity and are a promising model to investigate the (patho-)physiological relevancy of α-granules.
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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.