ArticleBlood2015
Defective PDI release from platelets and endothelial cells impairs thrombus formation in Hermansky-Pudlak syndrome.
Article in Blood, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 72 citations in OpenAlex.
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- Protein disulfide isomerase-A1 regulates intraplatelet reactive oxygen species-thromboxane AJournal of thrombosis and haemostasis : JTH · 2022Article
- Circulating Protein Disulfide Isomerase Is Associated with Increased Risk of Thrombosis inClinical cancer research : an official journal of the American Association for Cancer Research · 2021Article
- A zinc transporter, transmembrane protein 163, is critical for the biogenesis of platelet dense granules.Blood · 2021Article
- Inherited platelet diseases with normal platelet count: phenotypes, genotypes and diagnostic strategy.Haematologica · 2021Review
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- GPR56/ADGRG1 is a platelet collagen-responsive GPCR and hemostatic sensor of shear force.Proceedings of the National Academy of Sciences of the United States of America · 2020Article
- Mouse laser injury models: variations on a theme.Platelets · 2020Review
- Genetic variants associated with Hermansky-Pudlak syndrome.Platelets · 2020Article
- Protein disulfide isomerase in cardiovascular disease.Experimental & molecular medicine · 2020Review
- Altered platelet proteome in lupus anticoagulant (LA)-positive patients-protein disulfide isomerase and NETosis as new players in LA-related thrombosis.Experimental & molecular medicine · 2020Article
- Defective AP-3-dependent VAMP8 trafficking impairs Weibel-Palade body exocytosis in Hermansky-Pudlak Syndrome type 2 blood outgrowth endothelial cells.Haematologica · 2019Article
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7 authors at 2 institutions in 1 country.
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Abstract
Protein disulfide isomerase (PDI), secreted from platelets and endothelial cells after injury, is required for thrombus formation. The effect of platelet and endothelial cell granule contents on PDI-mediated thrombus formation was studied by intravital microscopy using a mouse model of Hermansky-Pudlak syndrome in which platelet dense granules are absent. Platelet deposition and fibrin generation were nearly absent, and extracellular PDI was significantly reduced in HPS6(-/-) mice after vascular injury. HPS6(-/-) platelets displayed impaired PDI secretion and impaired exocytosis of α granules, lysosomes, and T granules due to decreased sensitivity to thrombin, but these defects could be corrected by addition of subthreshold amounts of adenosine 5'-diphosphate (ADP). Human Hermansky-Pudlak syndrome platelets demonstrated similar characteristics. Infusion of wild-type platelets rescued thrombus formation in HPS6(-/-) mice. Human umbilical vein endothelial cells in which the HPS6 gene was silenced displayed impaired PDI secretion and exocytosis of Weibel-Palade bodies. Defective thrombus formation in Hermansky-Pudlak syndrome, associated with impaired exocytosis of residual granules in endothelial cells and platelets, the latter due to deficiency of ADP, is characterized by a defect in T granule secretion, a deficiency in extracellular PDI secretion, and impaired fibrin generation and platelet aggregation. Hermansky-Pudlak syndrome is an example of a hereditary disease whereby impaired PDI secretion contributes to a bleeding phenotype.
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