Lung Fibrosis





Pulmonary Fibrosis (PF)

Pulmonary Fibrosis is a disease in which tissue deep in the lungs become thick, stiff, and scarred over time.  Oxygen supply in the blood is reduced, resulting in perpetual shortness of breath.  Over 5 million people are affected by pulmonary fibrosis worldwide; and there is no cure.
 
There are over 140 known and countless idiopathic causes.  However, exposure to environmental pollutants and certain medications can cause the disease.  

Mice are the most common species used when studying pulmonary fibrosis. However, several animal models of lung fibrosis exist; ranging from mice to primates. 

Bleomycin is a commonly used chemotherapeutic agent that causes an acute lung injury response followed by lung fibrosis. Other approaches include asbestosis, instillation of silica, and transgenic models.
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Lung Fibrosis articles citing DSI in Google Scholar

Cystic Fibrosis (CF)

Cystic Fibrosis is an incurable and inherited disorder affecting multiple organ systems; including the lungs. Genetic mutations of a specific protein called cystic fibrosis transmembrane conductance regulator (CFTR) cause abnormally thick mucus to form in the lungs. The mucus blocks the airways, causing lung damage and making it difficult to breathe.  Although CF affects many areas of the body, complications and severe lung infections are the primary cause of death in most patients.
 
Animal models used to study CF typically focus on the use of mice with absent or mutant forms of the CFTR protein. Species including ferrets and pigs are also considered.

Pulmonary fibrosis and cystic fibrosis are two very different diseases. As such, the disease model studied often dictates the endpoints of interest. Although compliance and resistance are two commonly collected parameters, many supplemental endpoints can be calculated at the same time.

DSI solutions are helping researchers learn more about how to treat lung fibrosis.



FPRC - FinePointe resistance and compliance hardware helps researchers measure an animal’s airflow and lung pressure. PF models demonstrate a decrease in compliance as a result of stiff lungs. Obstructive CF models affect airway resistance.

Learn more about FinePointe resistance and compliance hardware.

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PFT - The pulmonary function testing system allows for a comprehensive assessment of the lungs.  All lung volumes and spirometry equivalent test results are completed in a matter of minutes; allowing for high throughput and quicker results.

Learn more about pulmonary function testing products from DSI.

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FinePointe Software – FinePointe software is powerful and easy-to-use for collecting, analyzing, and reporting life science data. Fully network-enabled, the smart design minimizes required user interaction while wizards walk users through necessary procedures.

Learn more about Buxco FinePointe software from DSI.

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References

Li M, Krishnaveni M, Li C, Zhou B, Xing Y, Banfalvi A, Li A, Lombardi V, Akbari O, Borok Z and Minoo P. “Epithelium-specific deletion of TGF-B receptor type II protects mice from bleomycin-induced pulmonary fibrosis.” Journal of Clinical Investigation. 2011;121:277-287.

Marcos V, Zhou-Suckow Z, Yildirim A, Bohla A, Hector A, Vitkov L, Krautgartner W, Stoiber W, Griese M, Eickelberg O, Mall M, Hartl D. “Free DNA in Cystic Fibrosis Airway Fluids Correlates with Airflow Obstruction.” Mediators of Inflammation. 2015.