Hermansky-Pudlak syndrome (HPS) is characterized by oculocutaneous albinism, bleeding
diathesis, and other variable symptoms. The bleeding diathesis has been attributed to δ …

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by
defective biogenesis of lysosome-related organelles. Clinical manifestations include a …

Hermansky-Pudlak syndrome (HPS) is a disorder of lysosome-related organelle biogenesis
that displays genetic locus heterogeneity. The eight known HPS proteins combine in …

The biogenesis of lysosome related organelles is defective in Hermansky-Pudlak syndrome
(HPS), a disorder characterized by oculocutaneous albinism and platelet dense granule …

Hermansky-Pudlak syndrome (HPS) is a rare, autosomal recessive disorder affecting
lysosome-related organelles (LRO), including dense platelet granules. HPS causes oculo …

Abstract Hermansky-Pudlak syndrome (HPS)− characterized by the distinct clinical
phenotypes of both oculocutaneous albinism and mild bleeding diathesis–is caused by …

Hermansky-Pudlak syndrome (HPS) is an inherited hemorrhagic disease affecting the
related subcellular organelles platelet dense granules, lysosomes, and melanosomes. The …

Introduction Oculocutaneous albinism (OCA) in combination with a platelet function defect
caused by a disturbed release reaction from platelet δ-granules (storage pool defect–SPD) …

Hermansky-Pudlak Syndrome (HPS) is an autosomal-recessive condition characterized by
oculocutaneous albinism and a bleeding diathesis due to absent platelet delta granules …

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 …