What question did this study set out to answer?

Investigate the longitudinal cellular interactions and molecular mechanisms that drive pulmonary fibrosis onset and progression in Hermansky-Pudlak syndrome.

February 22, 2025Communications BiologyOpen Access

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

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Authors

BBBrandon J. H. BanaschewskiChildren's Hospital of Philadelphia NMNigel S. MichkiChildren's Hospital of Philadelphia SSSneha SitaramanChildren's Hospital of Philadelphia

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Implication

Transcriptomic profiling reveals an age-dependent rise of inflammatory lung fibroblasts in Hermansky-Pudlak syndrome models, suggesting cellular targets to mitigate the fibrotic cascade.

Key Points

Investigate the longitudinal cellular interactions and molecular mechanisms that drive pulmonary fibrosis onset and progression in Hermansky-Pudlak syndrome.
Generated single-cell RNA sequencing (scRNA-seq) lung atlases across young and aged mouse models of Hermansky-Pudlak syndrome subtypes, including HPS1, HPS2, and double-mutant HPS1-2.
Assessed cellular crosstalk by co-culturing HPS2 alveolar type II epithelial cells with fibroblasts.
Performed scRNA-seq validation on lung tissue acquired from three human patients with HPS1.
Detected an age-dependent accumulation of SAA3+ inflammatory lung fibroblasts in HPS mouse models, particularly in double-mutant HPS1-2 mice exhibiting spontaneous fibrosis.
Showed that HPS2 alveolar type II epithelial cells stimulate inflammatory gene profiles in co-cultured fibroblasts, alongside elevated IL-1R1 expression in HPS1 fibroblasts.
Identified homologous populations of inflammatory fibroblasts and increased IL1R1 expression within lung tissue profiles of HPS1 patients.

Cite This Study

Banaschewski et al. (2025) studied this question.

synapsesocial.com/papers/6a7cece9a3cb31c5f9929366 https://doi.org/10.1038/s42003-025-07589-9

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Implication

Transcriptomic profiling reveals an age-dependent rise of inflammatory lung fibroblasts in Hermansky-Pudlak syndrome models, suggesting cellular targets to mitigate the fibrotic cascade.

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

Discussion

Member takes

Implication

Key Points

Cite This Study

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

Discussion

Member takes

Implication

Key Points

Cite This Study

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