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Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is a lung condition that brings about the formation of scar tissue and thickening in the lungs. The complexity of IPF is not to be understated, with its intricate interplays within the realms of pulmonary health. Our distinguished company Delving deeper into the realm of rare disease research and therapeutic. Through the marriage of our expertise and cutting-edge technology, we aim to help your research of IPF.

Introduction to IPF

Idiopathic pulmonary fibrosis (IPF) with a prevalence of 5.6 per 100,000 individuals, and predominantly affects middle-aged and elderly men. IPF is characterized by persistent dry cough, escalating fatigue, and progressive breathlessness (Fig.1). Individuals diagnosed with IPF commonly face potential complications, including hypertension, chronic obstructive pulmonary disease (COPD), emphysema, diabetes, and gastroesophageal reflux disease (GERD).

Fig.1 Comparison of healthy lung to IPF lung.Fig.1 Comparison of healthy lung to IPF lung. (Glass, D. S., et al., 2022)

Pathogenesis of IPF

This pathological through the aberrant deposition of scar tissue within the pulmonary, which for the disruption of normal lung function and the subsequent deprivation of adequate respiration. At the crux of IPF lies the activation of fibroblasts, immune cells, and cytokines—a cascade that sets the stage for inflammation and fibrosis, further exacerbating the plight of those afflicted by IPF (Fig.2).

Fig.2 Cellular senescence is a key mediator of IPF pathology.Fig.2 Cellular senescence is a key mediator of IPF pathology. (Moss, B. J., et al., 2022)

Therapeutics of IPF

Small Molecule Drugs Therapy

Small molecule drug therapies, including pirfenidone and nintedanib, targeting TGF-β and FGF/PDGF pathways, respectively, have shown efficacy in slowing down disease progression.

Gene Therapy

Gene therapy approaches, like the knockdown of human antigen R (HuR), have shown potential in reducing the induction of extracellular matrix proteins by TGF-β.

Monoclonal Antibodies Therapy

Monoclonal antibodies, such as pamrevlumab targeting CTGF and romilkimab targeting IL13 and IL4, have demonstrated promise in halting the fibrotic process and reducing lung scarring in IPF individuals.

Cell Therapy

Cell therapy involving mesenchymal stem cells (MSCs) has emerged as a promising therapeutic modality in IPF. By infusing bone marrow-derived MSCs into individuals, researchers aim to regulate the fibrosis process and promote lung repair.

Our Services

At the forefront of the realm stands our company, equipped with a team of dedicated professionals, cutting-edge technology, and a commitment to advancing IPF research. We can provide crucial resources such as animal models and therapeutics platforms, to enhance your understanding of IPF and foster the exploration of new therapeutic modalities and intervention strategies.

Platforms of IPF Therapy Development

Animal Models of IPF 

The animal models are valuable tools for studying the underlying mechanisms of IPF and for testing potential therapeutic interventions for the disease. Our company can provide chemical induction or genetic engineering animal models to support your research on the pathogenesis and preclinical therapeutic of IPF.

Chemical-induced Models
Bleomycin, lipopolysaccharide, silicon dioxide, or fluorescein isothiocyanate (FITC) are commonly used to induce lung fibrosis in animal models. They cause lung injury and inflammation leading to fibrosis, similar to the pathogenesis of IPF.
Optional Models
  • Bleomycin model
  • Lipopolysaccharide model
  • FITC model
  • Silicon dioxide model
Genetically Engineered Models
Transgenic or gene-editing techniques can be used to modify genes like TGF-β1 and SP-C in animals, leading to the development of progressive pulmonary fibrosis similar to IPF.
Optional Models
  • TGF-β1 transgenic model
  • SP-C mutant model
Physical-induced models
Radiation exposure can also cause lung injury and fibrosis in animal models, mimicking the features of IPF.
Optional Models Radiation-induced model
Optional Species Mice, Rats, Others

Our company focuses on providing comprehensive support throughout all stages of IPF research. From conceptualization to implementation, our seasoned researchers and advanced technology to support your pharmacokinetics research and drug safety evaluation, to guide and propel your research endeavors forward.

If you are interested in our services, please feel free to contact us for more details and quotation information of related services.

References

  • Glass, Daniel S, et al. "Idiopathic pulmonary fibrosis: Current and future treatment." The clinical respiratory journal 16.2 (2022): 84–96.
  • Moss, Benjamin J, et al. "Pathogenic Mechanisms Underlying Idiopathic Pulmonary Fibrosis." Annual review of pathology 17 (2022): 515–546.

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.

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