CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR PROTEIN EXPRESSION IN BRAIN

THE cystic fibrosis transmembrane conductance regulator protein (CFTR) has been identified in bovine brain clathrin-coated vesicles, rat bra in and a human neuroblastoma cell line using affinity-purified polyclo nal peptide antibodies against CFTR. Immunocytochemical staining of mu ltiple dendrites and soma of neurons of the diencephalon, midbrain, pe ns and medulla oblongata, has also been demonstrated. Whole cell lysat es and membranes derived from rat brain, neuroblastoma cells and bovin e brain clathrin-coated vesicles express the mature 150-165 kDa and 13 0 kDa unglycosylated forms of CFTR. The localization of CFTR to brain regions controlling homeostasis and energy expenditure may relate to t he pathogenesis of non-pulmonary manifestations of cystic fibrosis. CF TR expression in neurons and coated vesicles suggests a possible effec t on neuropeptide vesicle trafficking by mutant CFTR.