Development of Mouse Models for Cystic Fibrosis

Cystic fibrosis (CF) is a common deadly autosomal recessive disorder caused by mutations in the CFTR gene, the most common mutation (ΔF 508) occurs in about 70% of the CF chromosome. Dysfunction of the CFTR protein acting as an epithelial chloride channel located at the top is a classical CF such as salty sweat, pancreatic insufficiency, intestinal obstruction, male infertility and severe lung disease, with abnormalities characteristic of electrolyte transport Cause symptoms.

Cystic fibrosis is an autosomal recessive disorder - that is, two defective genes (one for each parent) are needed to develop the disease. The gene thought to cause cystic fibrosis is called cystic fibrosis transmembrane conductance regulator or CFTR. This gene is on chromosome 7. The severity and extent of this disease vary widely among patients with cystic fibrosis. Typically, this condition results in an increase in the chloride transport defect and trauma across epithelial cells and the viscosity of body secretions, particularly secretions from the respiratory tract (ie, the lungs, throat) and pancreas. As a result, patients are prone to pancreatic dysfunction and repeated chest infections

Most patients with cystic fibrosis develop lung disease at some point in their lifetime. "Respiratory complications are the leading cause of death in patients with cystic fibrosis" (CFF). In addition, cystic fibrosis patients are susceptible to certain antibiotic (CFF) resistant lung infections. This makes the lives of people with disease miserable. In nature, this disease can not survive to that extent. These breathing problems can easily lead to premature death. Antibiotics are used to help patients live. Cloning prevents transmission of diseases, only carriers and sick children without disease

Marten deVlieger was initially diagnosed with cystic fibrosis. This is a degenerative and incurable disease affecting the whole body, especially the lungs. "Life with cystic fibrosis is a continuous task." Most of us are said to be not 2 years old, 10 years old, 21 years old after birth ... to the current average CF life The living person thought that he shared the expectation of 42 years old, "I thought it might be", "He shared it, but Marten dreams of independence. During adolescence he We organized local dumps, started looking for metal scraps and parts and used it to make coarse heavy chest equipment to reflect the traditional mucus relaxation method of CF patients. ChestMaster 5000 was born.