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GRP78 overexpressing cells treated with tunicamycin or A23187 exhibited a reduced induction of endogenous GRP78 and GRP94 mRNAs compared to wild-type CHO cells. To further increase our understanding of the role of GRP78 in secretion, we have stably overexpressed GRP78 in Chinese hamster ovary (CHO) cells and examined the effect on protein secretion and the stress response. Some proteins which are destined for secretion transiently associate with GRP78. Many secretion incompetent proteins are found in stable association with GRP78 and are retained in the ER. GRP78 is a resident protein of the endoplasmic reticulum (ER) and a member of the glucose regulated protein (GRP) family. Suggest that ERp29 is a 4PBA-regulated ER chaperone that regulates wt CFTR biogenesis and can promote ΔF508-CFTR trafficking With endogenous ERp29 and overexpression of ERp29 led to increased ΔF508-CFTR expression at the plasma membrane. In IB3-1 cells, ΔF508-CFTR co-immunoprecipitated In CFBE41o-wt-CFTR cells, expression of and short circuit currents mediated byĬFTR decreased upon depletion of ERp29, as did maturation of newly synthesized CFTR. Wild-type CFTR plasma membrane expression. In Xenopus oocytes, ERp29 overexpression increased the functional expression of both wild-type and ΔF508-CFTR over 3-fold, and increased

Increased (~1.5 fold) in 4PBA-treated IB3-1 cells. ERp29 mRNA and protein expression was significantly Is a 4PBA-regulated ER chaperone that influences ΔF508-CFTR trafficking. Resident of the endoplasmic reticulum (ER) thought to be a novel molecular chaperone. One transcript found up-regulated by 4PBA encoded ERp29, a luminal On IB3-1 CF bronchiolar epithelial cells exposed to 4PBA. Other 4PBA-regulated proteins that might regulate ΔF508-CFTR trafficking, we performed a differential display RT-PCR screen The underlying mechanism is uncertain, but 4PBA modulates the expression of some cytosolic molecular chaperones. Sodium 4-Phenylbutyrate (4PBA) improves the intracellular trafficking of ΔF508-CFTR in Cystic Fibrosis (CF) epithelial cells.