First Idiopathic Pulmonary Fibrosis Mouse Model

Congratulations to Dr. Beers and his team for developing and phenotyping the first idiopathic pulmonary fibrosis mouse model!

Pulmonary fibrosis mouse model – why do we need them?

Mouse models are an important research tool for preclinical scientists. Alternatives including cells and computer simulations do exist, however they are not advanced enough to model all the interactions occurring within a living creature.

READ MORE: Workshop on Phenotyping Mouse Models of Human Lung Disease

What is idiopathic pulmonary fibrosis?

Idiopathic Pulmonary Fibrosis (IPF) is a life-threatening disease where scar tissue builds up around the lungs, making normal breathing difficult. Previous preclinical models were not able to correctly mimic the clinical features or pathogenesis of this degenerative disease. Consequently, leading to major challenges in the development of effective therapies to stabilize or improve lung function in people affected by IPF.

Study succeeds to develop IPF mouse model!

In their knock-in IPF mouse model, Dr. Beers’ group at the University of Pennsylvania demonstrated how mutations of a specific gene in the surfactant protein-C (SFTPC), led to spontaneous lung fibrosis. Results from the study support the hypothesis that epithelial cell dysfunction is an important component in the pathogenesis of this disease.1

Using the SCIREQ flexiVent, they measured pulmonary function including static lung compliance and airway resistance in mouse models at 6 weeks. Results show lung function is reduced as a consequence of SFTPC manipulation.

Pulmonary Fibrosis mouse model

Read more on Dynamic and Static Compliances with the flexiVent

Many researcher consider the flexiVent as the gold standard for in vivo lung function measurements. It captures more than the traditional resistance and compliance outcomes, offering lung volumes, lung partitioning, and flow limitation parameters as well. Above all, it achieves the highest sensitivity and reproducibility by precisely controlling experiment conditions.

1 Nureki SI, et al. Expression of mutant Sftpc in murine alveolar epithelia drives spontaneous lung fibrosis. J Clin Invest. https://doi. org/10.1172/JCI99287.

Read more Publications featuring the SCIREQ’s research systems.

Pulmonary Fibrosis mouse model

Empowering researchers

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