ABSTRACT
A couple of years aer the cloning of the human cystic brosis transmembrane conductance regulator gene (CFTR), the rst cystic brosis (CF) mouse had been generated1 using molecular techniques to “knockout” Cftr expression. Today, a large number of CF mouse models are available (see later). CF knockout mice do not spontaneously develop CF-like lung disease, but have been useful to highlight a number of important points including (1) showing a strong correlation between residual Cftr expression and survival based on gut disease, (2) highlighting the importance of strain dierences and thereby the potential eect of modier genes, and more recently (3) allowing the dissection of Cr eects in individual tissues and cell types using the conditional Cr knock-in and knockout mouse models.
