ArticleBlood2015
Defective release of α granule and lysosome contents from platelets in mouse Hermansky-Pudlak syndrome models.
Article in Blood, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
28 citing papers in PubMed, 54 citations in OpenAlex.
- Flow Cytometric Profiling Reveals Platelet Dysfunction and Impaired Immune Communication in Acute and Chronic Cerebrovascular Disease.Journal of clinical laboratory analysis · 2026Article
- Thrombin activity confinement and dense granule release drive the dynamics of arterial thrombus.PLoS computational biology · 2026Article
- Article
- Uncovering the role of the Hsp40 family member cysteine string protein-α in mouse platelets.Blood advances · 2025Article
- Review
- Murine Models of Familial Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Evolutionarily conserved role of hps1 in melanin production and blood coagulation in medaka fish.G3 (Bethesda, Md.) · 2022Article
- Article
- The secreted tyrosine kinase VLK is essential for normal platelet activation and thrombus formation.Blood · 2022Article
- A zinc transporter, transmembrane protein 163, is critical for the biogenesis of platelet dense granules.Blood · 2021Article
- Hemostasis vs. homeostasis: Platelets are essential for preserving vascular barrier function in the absence of injury or inflammation.Proceedings of the National Academy of Sciences of the United States of America · 2020Article
- Genetic variants associated with Hermansky-Pudlak syndrome.Platelets · 2020Article
- Mouse laser injury models: variations on a theme.Platelets · 2020Review
- Combined deficiency of RAB32 and RAB38 in the mouse mimics Hermansky-Pudlak syndrome and critically impairs thrombosis.Blood advances · 2019Article
- Platelet biology and functions: new concepts and clinical perspectives.Nature reviews. Cardiology · 2019Review
- SNARE-dependent membrane fusion initiates α-granule matrix decondensation in mouse platelets.Blood advances · 2018Article
- RGS10 shapes the hemostatic response to injury through its differential effects on intracellular signaling by platelet agonists.Blood advances · 2018Article
- The life cycle of platelet granules.F1000Research · 2018Review
- Platelet dense granules begin to selectively accumulate mepacrine during proplatelet formation.Blood advances · 2017Article
- The nuts and bolts of the platelet release reaction.Platelets · 2017Review
Corrections and comments
- Commented on by
Authors and funding
10 authors at 3 institutions in 3 countries.
Funding
Abstract
Hermansky-Pudlak syndrome (HPS) is characterized by oculocutaneous albinism, bleeding diathesis, and other variable symptoms. The bleeding diathesis has been attributed to δ storage pool deficiency, reflecting the malformation of platelet dense granules. Here, we analyzed agonist-stimulated secretion from other storage granules in platelets from mouse HPS models that lack adaptor protein (AP)-3 or biogenesis of lysosome-related organelles complex (BLOC)-3 or BLOC-1. We show that α granule secretion elicited by low agonist doses is impaired in all 3 HPS models. High agonist doses or supplemental adenosine 5'-diphosphate (ADP) restored normal α granule secretion, suggesting that the impairment is secondary to absent dense granule content release. Intravital microscopy following laser-induced vascular injury showed that defective hemostatic thrombus formation in HPS mice largely reflected reduced total platelet accumulation and affirmed a reduced area of α granule secretion. Agonist-induced lysosome secretion ex vivo was also impaired in all 3 HPS models but was incompletely rescued by high agonist doses or excess ADP. Our results imply that (1) AP-3, BLOC-1, and BLOC-3 facilitate protein sorting to lysosomes to support ultimate secretion; (2) impaired secretion of α granules in HPS, and to some degree of lysosomes, is secondary to impaired dense granule secretion; and (3) diminished α granule and lysosome secretion might contribute to pathology in HPS.
Indexed as
Identifiers
What OpenQuestion holds
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.