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F508 CFTR trafficking
Eudowood Division of Pediatric Respiratory Sciences, Department of Pediatrics, Johns Hopkins Medical Institutions, Baltimore, Maryland 21287-2533
The
F508 cystic fibrosis transmembrane conductance regulator (CFTR) is a
temperature-sensitive trafficking mutant that is detected as an
immature 160-kDa form (band B) in gel electrophoresis. The goal of this
study was to test the hypothesis that HSP70, a member of the 70-kDa
heat shock protein family, promotes F508 CFTR processing to the
mature 180-kDa form (band C). Both pharmacological and genetic
techniques were used to induce HSP70. IB3-1 cells were treated with
sodium 4-phenylbutyrate (4PBA) to promote maturation of F508 CFTR to
band C. A dose-dependent increase in band C and total cellular HSP70
was observed. Under these conditions, HSP70-CFTR complexes were
increased and 70-kDa heat shock cognate protein-CFTR complexes were
decreased. Increased F508 CFTR maturation was also seen after
transfection with an HSP70 expression plasmid and exposure to
glutamine, an inducer of HSP70. With immunofluorescence techniques, the
increased appearance of CFTR band C correlated with CFTR distribution
beyond the perinuclear regions. These data suggest that induction of
HSP70 promotes F508 CFTR maturation and trafficking.
cystic fibrosis; cystic fibrosis transmembrane conductance regulator; 70-kilodalton heat shock protein chaperones; phenylbutyrate; plasmid transfection; glutamine
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