Establishment of a humanized mouse model of pulmonary fibrosis for advancing drug validation strategies.

ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease characterized by excessive extracellular matrix deposition and limited therapeutic options. Conventional preclinical models, such as the bleomycin (BLM)-induced lung fibrosis (BILF) model in immunocompetent mice, fail to replicate the complexity of the human immune environment and are thus unsuitable for evaluating human-specific antibody and cell therapies. To overcome this limitation, we developed two humanized mouse models with functional human immune components: one engrafted with peripheral blood mononuclear cells (PBMCs) and the other with hematopoietic stem cells (HSCs). Pulmonary fibrosis was induced using BLM in both humanized models. In the PBMC-humanized model, human T cells accounted for 79.9% ± 9.7%

SUBMITTER: Hasan MN 

PROVIDER: S-EPMC12501095 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature