Cftr-KO Mouse

Cystic Fibrosis (CF) is an autosomal recessive disorder causing severe damage to the lungs, digestive system, and other organs. It thickens mucus, sweat, and digestive fluids, blocking ducts and channels. The disease manifests as a persistent cough, hyperinflation of lung lobes, chronic nasal congestion, headaches, sleep disorders, digestive and reproductive system disorders, and nutritional and growth development disorders. CF is caused by mutations in the CF-transmembrane conductance regulator (CFTR) gene, which encodes a cAMP-dependent chloride ion channel protein. Abnormal CFTR function can cause transmembrane transport disorders of chloride ions and bicarbonate, leading to mucus obstruction in exocrine glands, and affecting respiration, digestion, endocrine, and reproduction ^[1-2].

Cftr-KO mice are a gene-knockout (KO) model. Using gene-editing technology, exons 5-6 of the Cftr gene in mice have been knocked out. This model can be used for research on the pathogenic mechanism of cystic fibrosis and the development of related treatment methods. Homozygous Cftr-KO mice start to die at 2 weeks of age, and prophylactic PEG treatment can improve their survival rate ^[3]. This strain requires feeding with intestinal cleansers to maintain survival after 3 weeks of age.