Emergence of inflammatory fibroblasts with aging in Hermansky-Pudlak syndrome associated pulmonary fibrosis.

Brandon J H Banaschewski, Sylvia N Michki, Sneha Sitaraman, Ruby Pan, Joanna Y Wang, Dominique Stewart, Mary Kate Goldy, Susan M Lin, Edward Cantu, Jeremy B Katzen, Maria C Basil, Amir M Emtiazjoo, Jamie L Todd, Jason J Gokey, Jonathan A Kropski, David B Frank, Jarod A Zepp, Lisa R Young

Division of Pulmonary and Sleep Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA.Division of Pulmonary and Sleep Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA. · pubmed

Paper

Abstract

The longitudinal cellular interactions that drive pulmonary fibrosis are not well understood. To investigate the disease underpinnings associated with fibrosis onset and progression, we generated a scRNA-seq atlas of lungs from young and aged mouse models of multiple subtypes of Hermansky-Pudlak syndrome (HPS), a collection of rare autosomal recessive diseases associated with albinism, platelet dysfunction, and pulmonary fibrosis. We have identified an age-dependent increase in SAA3

The following analysis is generated by AI and may contain errors. It is separate from the authors’ abstract and is not peer review.

Research summary

The paper identifies an age-dependent increase in inflammatory fibroblasts in pulmonary fibrosis associated with Hermansky-Pudlak syndrome. This research is relevant as it explores cellular mechanisms linked to aging and disease progression, potentially contributing to understanding age-related pathologies.

Author of this paper? Send a correction.