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Journal of Clinical Investigation, ISSN 0021-9738, 08/2015, Volume 125, Issue 8, pp. 3178 - 3192
Hermansky-Pudlak syndrome (HPS) comprises a group of inherited disorders caused by mutations that alter the function of lysosome-related organelles. Pulmonary... 
MEDICINE, RESEARCH & EXPERIMENTAL | TISSUE RESPONSES | EPITHELIAL-CELLS | GRANULOMATOUS COLITIS | INFLAMMATION | MOUSE MODEL | IL-13R-ALPHA-2 | LYSOSOME-RELATED ORGANELLES | IL-13 | IDIOPATHIC PULMONARY-FIBROSIS | BREAST REGRESSION PROTEIN-39 | Humans | Male | Pulmonary Fibrosis - blood | Pulmonary Fibrosis - genetics | Receptors, Prostaglandin - metabolism | Adipokines - blood | Chitinase-3-Like Protein 1 | Hermanski-Pudlak Syndrome - blood | Respiratory Mucosa - pathology | Receptors, Prostaglandin - genetics | Adipokines - genetics | Adult | Female | Pulmonary Fibrosis - etiology | Disease Models, Animal | Glycoproteins - genetics | Interleukin-13 Receptor alpha2 Subunit - metabolism | Glycoproteins - blood | Pulmonary Fibrosis - pathology | Biomarkers - blood | Mice, Knockout | Hermanski-Pudlak Syndrome - genetics | Lectins - blood | Animals | Hermanski-Pudlak Syndrome - complications | Interleukin-13 Receptor alpha2 Subunit - genetics | Hermanski-Pudlak Syndrome - pathology | Lectins - genetics | Mice | Respiratory Mucosa - metabolism | Receptors, Immunologic - genetics | Receptors, Immunologic - metabolism | Apoptosis | Enzymes | Pulmonary fibrosis | Gene mutations | Physiological aspects | Development and progression | Hydrolases | Genetic aspects | Research | Health aspects | Proteins | Studies | Chitinase | Plasma | Laboratories | Rodents | Lung diseases | Mutation | Defects
Journal Article
Blood, ISSN 0006-4971, 2015, Volume 125, Issue 10, pp. 1633 - 1642
Journal Article
Journal Article
Journal Article
Blood, ISSN 0006-4971, 2015, Volume 125, Issue 10, pp. 1623 - 1632
Journal Article
Clinics in Chest Medicine, ISSN 0272-5231, 09/2016, Volume 37, Issue 3, pp. 505 - 511
Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diatheses, granulomatous colitis,... 
Biogenesis of lysosome-related organelle complex | Pulmonary fibrosis | Adaptor protein 3 | Albinism | Hermansky-Pudlak syndrome | Interstitial lung disease | MUTATION SCREEN REVEALS | APOPTOSIS | FORM | SUSCEPTIBILITY | COLITIS | RESPIRATORY SYSTEM | INFLAMMATION | MOUSE MODEL | LUNG-DISEASE | PULMONARY-FIBROSIS | IMMUNODEFICIENCY | Epistaxis - etiology | Hemorrhagic Disorders - physiopathology | Pulmonary Veins - abnormalities | Gastrointestinal Diseases - physiopathology | Humans | Gastrointestinal Diseases - etiology | Hypertension, Pulmonary - physiopathology | Telangiectasis - physiopathology | Epistaxis - physiopathology | Arteriovenous Malformations - physiopathology | Intracranial Arteriovenous Malformations - physiopathology | Albinism, Oculocutaneous - physiopathology | Hemorrhagic Disorders - complications | Liver Diseases - etiology | Intracranial Arteriovenous Malformations - etiology | Crohn Disease - etiology | Pulmonary Fibrosis - etiology | Albinism - physiopathology | Blood Coagulation Disorders - etiology | Arteriovenous Malformations - etiology | Pulmonary Artery - abnormalities | Albinism - complications | Albinism, Oculocutaneous - etiology | Telangiectasis - etiology | Hermanski-Pudlak Syndrome - complications | Pulmonary Fibrosis - physiopathology | Hermanski-Pudlak Syndrome - physiopathology | Crohn Disease - physiopathology | Liver Diseases - physiopathology | Hypertension, Pulmonary - etiology | Blood Coagulation Disorders - physiopathology
Journal Article
Journal Article
Journal of Thrombosis and Haemostasis, ISSN 1538-7933, 04/2016, Volume 14, Issue 4, pp. 839 - 849
Journal Article
Journal Article
Journal Article