Cystic Fibrosis (CF) is a mono-genetic disease caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Although the disease results in many manifestations, it is consistently characterized by chronic lung infection, poor growth, exocrine pancreatic insufficiency, intestinal obstruction and malabsorption, reduced fertility, and shortened lifespan. Understanding this multi-systemic disease requires the use of animal models that provide the opportunity to study complex physiologic processes in the context of a living organism. As there are a multitude of processes disrupted in CF, animal models that can allow one to approach these processes in a tractable manner are crucial.
Learn more about Cystic Fibrosis from the Cystic Fibrosis Foundation
This website is intended to serve as a resource about existing animal models of cystic fibrosis that are available to the research community. Although this site is not intended to contain all existing information about every single CF animal model published, this site aims to give a general overview of the CF animal models, provide links to suggested literature, and provide contact information for laboratories and cores that can assist in obtaining each CF animal model.
Pigs share many similarities to humans including body size, anatomical features, and physiology.
Ferrets share anatomical, cellular, metabolic, and physiological features with humans, including susceptibility to many human lung pathogens and pancreatic disease.
The anatomy, physiology, and development of the sheep respiratory system is similar to humans and its development, anatomy, and physiology is well characterized.
Rabbits have a long history in medical research, and antibodies are readily available for immunological studies.
Rats are large enough that surgeries, including transplants, are viable, but small enough that populations can be economically maintained. Rats are the traditional model for physiologic, pharmacologic, and toxicologic studies.
Mice are easily bred, genetically modified, and economical to maintain. There are genetic and physiological similarities between mice and humans. Genetic modification of mice is straightforward, which has led to the creation of a vast array of tools for research unmatched by any other mammalian model.
Zebrafish are optically transparent and genetically tractable, allowing the study of gene function in live animals using fluorescent reporters and organ-specific assays.