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논문분류	춘계학술대회 초록집
제목	Regulation of Epithelial Anion Transport: Role of PDZ-based adaptors and rescue by unconventional secretion
저자	Min goo LEE
출판정보	2017; 2017(1):
키워드	Anion Transporter, ABCC, SLC26, PDZ, Unconventional Secretion
초록	Anion transporters play a pivotal role in the transepithelial transport of small molecules in diverse body compartments including renal systems. Among many anion transporting proteins, the C subfamily of ATP-binding cassette (ABCC) and the solute carrier 26 (SLC26) transporters have received much attention because mutations affecting these transporter genes are the underlying causes for a large number of human diseases including cystic fibrosis, Pendred syndrome, and congenital nephrolithiasis. As a membrane protein, appropriate cell surface expression of these transporters is critical for physiological function of transporting proteins. I will focus on two mechanisms that regulate or enhance the surface expression of ABCC and SLC26 transporters. 1. Regulation by PDZ-based adaptors: Many of these transporters have a class 1 PDZ-biding motif at the COOH-termini and are regulated by PDZ-based adaptors. We have shown that several PDZ adaptors, such as Shank2 and NHERF3, regulate the cell surface expression of anion transporters including CFTR and MRP4. In particular, NHERF3-MRP4 interaction is critical for the MRP4-mediated systemic clearance of the antiviral agent adefovir in the kidney. 2. Rescue by unconventional secretion: Most disease-causing mutations of ABCC and SLC26 evoke protein folding and trafficking defects that prevent surface expression of mutant proteins. As a signal peptide-containing protein, these transporters reach the plasma membrane through the conventional ER-to-Golgi pathway. However, evidence suggests that these proteins also can reach the cell surface by unconventional trafficking pathways that bypass the Golgi. Recently, our group found that the selective activation of this unconventional trafficking pathway would be a potential therapeutic strategy for the treatment of diseases arising from the trafficking defects of misfolded proteins.
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