Cystic fibrosis (CF) often results from the failure of the CFTR chloride channel to hook up with the COPII machinery, based on results from Wang et al. (page 65).
The most common form of CF occurs in response to loss of phenylalanine 508 (F508) in CFTR, resulting in a failure to be exported from the ER. F508 deletion leads to protein misfolding and ER-associated degradation (ERAD). Many groups thus hope to interfere with ERAD to treat CF. But Wang et al. find that the solution may lie elsewhere. “We don't think less degradation,” says group leader William Balch. “We think more export.”
Export to the Golgi is required for CFTR to get to its functional location on the cell surface. The authors find that this export relies on a conserved di-acidic motif that must be properly presented to Sec24, a component of the...
