Cystic Fibrosis
Electron microscopy images of CF lungs
What is Cystic fibrosis?
Cystic fibrosis is caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) channel, causing viscous secretions. Clinically this leads to difficulties breathing and frequent lung infections. The channel has also recently been implicated to function in lymphocytes and may be playing a role in calcium dependent activation of T-cells. It is thought that the dysfunction of the channel in T-cells may have some consequences with predisposes the patients to allergy and reducing their ability to fight lung infections.
Therapeutic strategy
We use gene therapy to express CFTR in the lung or correct the T-cell phenotype by gene therapy or calcium channel modulators.
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