Brandon J. Banaschewski

Children's Hospital of Philadelphia

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Co-Authors

David B. Frank

University of Pennsylvania

Lisa R. Young

Johns Hopkins Medicine

Jarod A. Zepp

Children's Hospital of Philadelphia

J. Wang

Chungbuk National University

N.S. Michki

Children's Hospital of Philadelphia

Susan Lin

University of Pennsylvania

Jeremy Katzen

University of Pennsylvania

Mary Kate Goldy

Children's Hospital of Philadelphia

Nigel S. Michki

Children's Hospital of Philadelphia

Sneha Sitaraman

Roivant Sciences (United States)

Cooperative Institutions

Children's Hospital of Philadelphia

University of California, San Diego

University of Pennsylvania

Hospital of the University of Pennsylvania

Philadelphia University

Pulmonary and Allergy Associates

Vanderbilt University

Cincinnati Children's Hospital Medical Center

California University of Pennsylvania

National Jewish Health

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Excess neuropeptides in lung signal through endothelial cells to impair gas exchange

Developmental Cell (2022)

Jinhao Xu Le Xu Pengfei Sui Jiyuan Chen Esteban A. Moya

10.1016/j.devcel.2022.02.023

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Citations (24)

Fibroblasts From Hermansky-pudlak Syndrome Mice Contribute to Altered Growth in Alveolar Organoids

Brandon J. Banaschewski Pedro Mário Pan N.S. Michki Prashant Chandrasekaran Mary Kate Goldy

Organoid

Hermansky–Pudlak syndrome

10.1164/ajrccm-conference.2023.207.1_meetingabstracts.a4693

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Citations (1)

Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome pulmonary fibrosis

bioRxiv (Cold Spring Harbor Laboratory) (2023)

Joanna Y. Wang Nigel S. Michki Sneha Sitaraman Brandon J. Banaschewski Susan Lin

Hermansky-Pudlak syndrome (HPS) is a genetic disorder of endosomal protein trafficking associated with pulmonary fibrosis in specific subtypes, including HPS-1 and HPS-2. Single mutant HPS1 and HPS2 mice display increased fibrotic sensitivity while double mutant HPS1/2 mice exhibit spontaneous fibrosis with aging, which has been attributed to HPS mutations in alveolar epithelial type II (AT2) cells. We utilized HPS mouse models and human lung tissue to investigate mechanisms of AT2 cell dysfunction driving fibrotic remodeling in HPS. Starting at 8 weeks of age, HPS mice exhibited progressive loss of AT2 cell numbers. HPS AT2 cell was impaired ex vivo and in vivo. Incorporating AT2 cell lineage tracing in HPS mice, we observed aberrant differentiation with increased AT2-derived alveolar epithelial type I cells. Transcriptomic analysis of HPS AT2 cells revealed elevated expression of genes associated with aberrant differentiation and p53 activation. Lineage tracing and modeling studies demonstrated that HPS AT2 cells were primed to persist in a Krt8+ reprogrammed transitional state, mediated by p53 activity. Intrinsic AT2 progenitor cell dysfunction and p53 pathway dysregulation are novel mechanisms of disease in HPS-related pulmonary fibrosis, with the potential for early targeted intervention before the onset of fibrotic lung disease.

Hermansky–Pudlak syndrome

Progenitor

10.1101/2023.06.17.545390

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Citations (1)

Age-dependent Loss of Alveolar Type II Cells and Impaired Proliferation in a Murine Model of Hermansky-Pudlak Syndrome-related Pulmonary Fibrosis

J. Wang N.S. Michki Prashant Chandrasekaran R. Pan Brandon J. Banaschewski

Hermansky–Pudlak syndrome

10.1164/ajrccm-conference.2023.207.1_meetingabstracts.a2668

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Citations (1)

Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome

JCI Insight (2024)

Joanna Y. Wang Nigel S. Michki Sneha Sitaraman Brandon J. Banaschewski Reshma Jamal

Hermansky-Pudlak syndrome (HPS) is a genetic disorder of endosomal protein trafficking associated with pulmonary fibrosis in specific subtypes, including HPS-1 and HPS-2. Single mutant HPS1 and HPS2 mice display increased fibrotic sensitivity while double mutant HPS1/2 mice exhibit spontaneous fibrosis with aging, which has been attributed to HPS mutations in alveolar epithelial type II (AT2) cells. We utilized HPS mouse models and human lung tissue to investigate mechanisms of AT2 cell dysfunction driving fibrotic remodeling in HPS. Starting at 8 weeks of age, HPS mice exhibited progressive loss of AT2 cell numbers. HPS AT2 cell function was impaired ex vivo and in vivo. Incorporating AT2 cell lineage tracing in HPS mice, we observed aberrant differentiation with increased AT2-derived alveolar epithelial type I cells. Transcriptomic analysis of HPS AT2 cells revealed elevated expression of genes associated with aberrant differentiation and p53 activation. Lineage tracing and organoid modeling studies demonstrated that HPS AT2 cells were primed to persist in a Krt8+ reprogrammed transitional state, mediated by p53 activity. Intrinsic AT2 progenitor cell dysfunction and p53 pathway dysregulation are novel mechanisms of disease in HPS-related pulmonary fibrosis, with the potential for early targeted intervention before the onset of fibrotic lung disease.

Hermansky–Pudlak syndrome

Progenitor

10.1172/jci.insight.183483

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Citations (0)