CB1R and iNOS are distinct players promoting pulmonary fibrosis in Hermansky–Pudlak syndrome

CB1R and iNOS are distinct players promoting pulmonary fibrosis in Hermansky–Pudlak syndrome

Abstract Hermansky–Pudlak syndrome (HPS) is a rare genetic disorder which, in its most common and severe form, HPS‐1, leads to fatal adult‐onset pulmonary fibrosis (PF) with no effective treatment. We evaluated the role of the endocannabinoid/CB1R system and inducible nitric oxide synthase (iNOS) fo...

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Bibliographic Details
Main Authors: Resat Cinar, Joshua K. Park, Charles N. Zawatsky, Nathan J. Coffey, Steven P. Bodine, Jasmina Abdalla, Tadafumi Yokoyama, Tony Jourdan, Lindsey Jay, Mei Xing G. Zuo, Kevin J. O'Brien, Junfeng Huang, Ken Mackie, Asaf Alimardanov, Malliga R. Iyer, William A. Gahl, George Kunos, Bernadette R. Gochuico, May Christine V. Malicdan
Format: Article
Language:English
Published: Wiley 2021-07-01
Series:Clinical and Translational Medicine
Subjects:
endocannabinoids
fibrosis
lung disease
polypharmacology
rare disease
Online Access:https://doi.org/10.1002/ctm2.471

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