| http://purl.uniprot.org/citations/25477496 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/25477496 | http://www.w3.org/2000/01/rdf-schema#comment | "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."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.org/dc/terms/identifier | "doi:10.1182/blood-2014-07-586727"xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Wu J."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Wang Y."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Brass L.F."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Marks M.S."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Meng R."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Kowalska M.A."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Poncz M."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Abrams C.S."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Stalker T.J."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/author | "Harper D.C."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/name | "Blood"xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/pages | "1623-1632"xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/title | "Defective release of alpha granule and lysosome contents from platelets in mouse Hermansky-Pudlak syndrome models."xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://purl.uniprot.org/core/volume | "125"xsd:string |
| http://purl.uniprot.org/citations/25477496 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/25477496 |
| http://purl.uniprot.org/citations/25477496 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/25477496 |
| http://purl.uniprot.org/uniprot/#_A1L371-mappedCitation-25477496 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/25477496 |
| http://purl.uniprot.org/uniprot/#_A0A338P6V5-mappedCitation-25477496 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/25477496 |
| http://purl.uniprot.org/uniprot/#_A2VDG2-mappedCitation-25477496 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/25477496 |
| http://purl.uniprot.org/uniprot/#_Q9R0C0-mappedCitation-25477496 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/25477496 |
| http://purl.uniprot.org/uniprot/#_P23440-mappedCitation-25477496 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/25477496 |