ArticleBlood2012
The VPS33B-binding protein VPS16B is required in megakaryocyte and platelet α-granule biogenesis.
Article in Blood, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 84 citations in OpenAlex.
- Potential Mechanisms of Platelet Dysfunction and Bleeding in Acid Sphingomyelinase Deficiency.Cells · 2026Review
- Beyond Hemostasis: Platelets' Multifaceted Functions in Immune Responses.Life (Basel, Switzerland) · 2026Review
- The primate-specific Nedd4-1(NE) localizes to late endosomes in response to amino acids to suppress autophagy.Nature communications · 2025Article
- The winding road to platelet α-granules.Frontiers in cell and developmental biology · 2025Review
- [Rare VPS33B gene mutation combined with GP1BA mutation causes severe decrease in plasma VWF levels: a case report and literature review].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2024Review
- Sorting nexin 24 is required for α-granule biogenesis and cargo delivery in megakaryocytes.Haematologica · 2022Article
- Syntaxin 12 and COMMD3 are new factors that function with VPS33B in the biogenesis of platelet α-granules.Blood · 2022Article
- VPS33B interacts with NESG1 to suppress cell growth and cisplatin chemoresistance in ovarian cancer.Cancer science · 2021Article
- Article
- Hypersensitivity of Vps33B mutant flies to non-pathogenic infections is dictated by aberrant activation of p38b MAP kinase.Traffic (Copenhagen, Denmark) · 2020Article
- Megakaryocytes package contents into separate α-granules that are differentially distributed in platelets.Blood advances · 2019Article
- Mechanism of platelet α-granule biogenesis: study of cargo transport and the VPS33B-VPS16B complex in a model system.Blood advances · 2019Article
- Molecular mechanisms of bleeding disorderassociated GFI1BHaematologica · 2019Article
- The road to lysosome-related organelles: Insights from Hermansky-Pudlak syndrome and other rare diseases.Traffic (Copenhagen, Denmark) · 2019Review
- Unlocking the Molecular Secrete(s) of α-Granule Biogenesis.Arteriosclerosis, thrombosis, and vascular biology · 2018Article
- Sorting machineries: how platelet-dense granules differ from α-granules.Bioscience reports · 2018Review
- Article
- Proteomic and Biochemical Comparison of the Cellular Interaction Partners of Human VPS33A and VPS33B.Journal of molecular biology · 2018Article
- The life cycle of platelet granules.F1000Research · 2018Review
- VPS18 recruits VPS41 to the human HOPS complex via a RING-RING interaction.The Biochemical journal · 2017Article
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Authors and funding
13 authors at 5 institutions in 3 countries.
Funding
Abstract
Patients with platelet α or dense δ-granule defects have bleeding problems. Although several proteins are known to be required for δ-granule development, less is known about α-granule biogenesis. Our previous work showed that the BEACH protein NBEAL2 and the Sec1/Munc18 protein VPS33B are required for α-granule biogenesis. Using a yeast two-hybrid screen, mass spectrometry, coimmunoprecipitation, and bioinformatics studies, we identified VPS16B as a VPS33B-binding protein. Immunoblotting confirmed VPS16B expression in various human tissues and cells including megakaryocytes and platelets, and also in megakaryocytic Dami cells. Characterization of platelets from a patient with arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome containing mutations in C14orf133 encoding VPS16B revealed pale-appearing platelets in blood films and electron microscopy revealed a complete absence of α-granules, whereas δ-granules were observed. Soluble and membrane-bound α-granule proteins were reduced or undetectable, suggesting that both releasable and membrane-bound α-granule constituents were absent. Immunofluorescence microscopy of Dami cells stably expressing GFP-VPS16B revealed that similar to VPS33B, GFP-VPS16B colocalized with markers of the trans-Golgi network, late endosomes and α-granules. We conclude that VPS16B, similar to its binding partner VPS33B, is essential for megakaryocyte and platelet α-granule biogenesis.
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Registered trials
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.