In Vivo Model Development for Idiopathic Pulmonary Fibrosis

Protheragen offers comprehensive in vivo animal model development services for Idiopathic Pulmonary Fibrosis (IPF), supporting preclinical research and therapeutic evaluation. Leveraging a diverse portfolio of validated models in multiple species, we enable clients to investigate disease mechanisms and assess the efficacy of novel interventions with scientific rigor and translational relevance.

Idiopathic Pulmonary Fibrosis is a progressive and fatal lung disease characterized by excessive scarring and functional decline. Robust animal models are essential for unraveling disease pathogenesis and for evaluating the safety and efficacy of emerging therapies. At Protheragen, we utilize a broad spectrum of species and strains—including Macaca fascicularis (Cynomolgus monkey), Mus musculus (various mouse strains such as C57BL/6, Balb/c, B6.129S4-Park2tm1Shn/J, CB17 Beige, and Kunming), and Rattus norvegicus (rat strains such as Sprague Dawley, Wistar, Brown Norway, and CD). These models recapitulate key features of human IPF, including fibrotic remodeling, immune dysregulation, and impaired lung function, thereby providing a robust platform for translational research.

Chemically-Induced Models

Chemically-induced models are established by administering agents such as bleomycin sulfate, quartz, or monocrotaline via intratracheal, intranasal, or systemic routes to induce pulmonary fibrosis. These models are widely used in mice (e.g., C57BL/6, Balb/c, Kunming), rats (e.g., Sprague Dawley, Wistar, Brown Norway), and non-human primates (Macaca fascicularis). Key advantages include rapid induction of fibrosis, reproducibility, and well-characterized progression that mirrors critical aspects of human IPF. Applications include mechanistic studies, therapeutic efficacy testing, and biomarker discovery.

Genetically-Modified Models

Genetically-modified models employ knockout, conditional knockout, or transgenic strategies to target genes implicated in fibrosis (e.g., Prkn, Rag2, Mir34a, Trp53, Chit1, Ercc1, Mir92-1, Nedd4l, HAS2, TGFB1). These models allow for the investigation of gene function in disease onset and progression, and can be combined with chemical induction or xenografting of human cells. Advantages include precise mechanistic interrogation, the ability to model genetic susceptibility, and suitability for long-term studies. Primary applications are in target validation, mechanistic elucidation, and evaluation of gene-targeted therapies.

Xenograft and Allograft Models

Xenograft and allograft models involve the transplantation of human or murine cells (such as airway basal cells or lung fibroblasts from IPF patients, or Lewis lung carcinoma cells) into immunodeficient or genetically-engineered mice (e.g., Rag2 knockout, CB17 Beige). These models enable the study of human cell behavior in an in vivo context and facilitate the assessment of human-specific therapeutic responses. Advantages include increased translational relevance and the ability to explore cell-based therapies or human disease mechanisms. Applications include preclinical efficacy testing, cell therapy development, and humanized disease modeling.

Radiation- and Biological Agent-Induced Models

These models utilize non-chemical insults, such as thoracic irradiation or biological agents like lipopolysaccharide (LPS), to induce lung fibrosis. Radiation-induced models are particularly valuable for studying chronic fibrotic changes and the impact of environmental exposures, while LPS models are used to investigate the interplay between inflammation and fibrosis. Advantages include the ability to mimic specific clinical scenarios and to dissect the contribution of non-chemical factors. Applications include research on environmental and occupational risk factors, inflammation-driven fibrosis, and combination therapy evaluation.

Protheragen delivers a full spectrum of in vivo IPF model development and testing services, from model selection and protocol optimization to comprehensive efficacy and safety assessment. Key efficacy endpoints include histopathological evaluation (e.g., Ashcroft scoring, Masson's trichrome staining), lung function testing (e.g., compliance, resistance), molecular and cellular biomarker analysis (e.g., hydroxyproline content, cytokine profiling, gene expression), and in vivo imaging (e.g., micro-CT). Our analytical capabilities encompass immunohistochemistry, flow cytometry, ELISA, qPCR, and next-generation sequencing. Rigorous quality control measures are embedded throughout, including standardized induction protocols, randomization, blinded assessment, and reproducible data reporting, ensuring high scientific integrity and regulatory compliance.

Partnering with Protheragen provides you with access to a diverse array of validated IPF animal models, expert scientific guidance, and end-to-end preclinical support. Our commitment to quality, innovation, and client collaboration accelerates your IPF research and therapeutic development. Contact us today to discuss your project needs and discover how Protheragen can advance your IPF program from concept to clinic.