Search Results - "Frizzell, Raymond"

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations by Veit, Gudio, Avramescu, Radu G, Chiang, Annette N, Houck, Scott A, Cai, Zhiwei, Peters, Kathryn W, Hong, Jeong S, Pollard, Harvey B, Guggino, William B, Balch, William E, Skach, William R, Cutting, Garry R, Frizzell, Raymond A, Sheppard, David N, Cyr, Douglas M, Sorscher, Eric J, Brodsky, Jeffrey L, Lukacs, Gergely L

“…More than 2000 mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) have been described that confer a range of molecular cell biological…”
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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770 by Van Goor, Fredrick, Hadida, Sabine, Grootenhuis, Peter D.J, Burton, Bill, Cao, Dong, Neuberger, Tim, Turnbull, Amanda, Singh, Ashvani, Joubran, John, Hazlewood, Anna, Zhou, Jinglan, McCartney, Jason, Arumugam, Vijayalaksmi, Decker, Caroline, Yang, Jennifer, Young, Chris, Olson, Eric R, Wine, Jeffery J, Frizzell, Raymond A, Ashlock, Melissa, Negulescu, Paul

“…Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), a protein kinase A…”
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Divergent signaling via SUMO modification: potential for CFTR modulation by Ahner, Annette, Gong, Xiaoyan, Frizzell, Raymond A

“…The cystic fibrosis transmembrane conductance regulator (CFTR) is generally responsible for the cAMP/PKA regulated anion conductance at the apical membranes of…”
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Cystic fibrosis transmembrane conductance regulator degradation: cross‐talk between the ubiquitylation and SUMOylation pathways by Ahner, Annette, Gong, Xiaoyan, Frizzell, Raymond A.

“…Defining the significant checkpoints in cystic fibrosis transmembrane conductance regulator (CFTR) biogenesis should identify targets for therapeutic…”
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Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions by Bozoky, Zoltan, Krzeminski, Mickael, Muhandiram, Ranjith, Birtley, James R, Al-Zahrani, Ateeq, Thomas, Philip J, Frizzell, Raymond A, d, Robert C, man-Kay, Julie D

“…Intrinsically disordered proteins play crucial roles in regulatory processes and often function as protein interaction hubs. Here, we present a detailed…”
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Comparative analyses of long non-coding RNA profiles in vivo in cystic fibrosis lung airway and parenchyma tissues by Kumar, Parameet, Sen, Chaitali, Peters, Kathryn, Frizzell, Raymond A, Biswas, Roopa

“…Recent advances in the functional analyses of endogenous non-coding RNA (ncRNA) molecules, including long non-coding RNAs (LncRNAs), have provided a new…”
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Different SUMO paralogues determine the fate of wild-type and mutant CFTRs: biogenesis versus degradation by Gong, Xiaoyan, Liao, Yong, Ahner, Annette, Larsen, Mads Breum, Wang, Xiaohui, Bertrand, Carol A, Frizzell, Raymond A

“…A pathway for cystic fibrosis transmembrane conductance regulator (CFTR) degradation is initiated by Hsp27, which cooperates with Ubc9 and binds to the common…”
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Physiology of epithelial chloride and fluid secretion by Frizzell, Raymond A, Hanrahan, John W

“…Epithelial salt and water secretion serves a variety of functions in different organ systems, such as the airways, intestines, pancreas, and salivary glands…”
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The CFTR trafficking mutation F508del inhibits the constitutive activity of SLC26A9 by Bertrand, Carol A, Mitra, Shalini, Mishra, Sanjay K, Wang, Xiaohui, Zhao, Yu, Pilewski, Joseph M, Madden, Dean R, Frizzell, Raymond A

“…Several members of the SLC26A family of anion transporters associate with CFTR, forming complexes in which CFTR and SLC26A functions are reciprocally…”
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Cystic Fibrosis Transmembrane Conductance Regulator Folding Mutations Reveal Differences in Corrector Efficacy Linked to Increases in Immature Cystic Fibrosis Transmembrane Conductance Regulator Expression by Peters, Kathryn W., Gong, Xiaoyan, Frizzell, Raymond A.

“…Background: Most cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that lead to protein misfolding…”
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Separating the contributions of SLC26A9 and CFTR to anion secretion in primary human bronchial epithelia by Larsen, Mads B, Choi, Jeannie J, Wang, Xiaohui, Myerburg, Michael M, Frizzell, Raymond A, Bertrand, Carol A

“…Aberrant anion secretion across the bronchial epithelium is associated with airway disease, most notably in cystic fibrosis. Although the cystic fibrosis…”
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Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway by Ahner, Annette, Gong, Xiaoyan, Schmidt, Bela Z, Peters, Kathryn W, Rabeh, Wael M, Thibodeau, Patrick H, Lukacs, Gergely L, Frizzell, Raymond A

“…Small heat shock proteins (sHsps) bind destabilized proteins during cell stress and disease, but their physiological functions are less clear. We evaluated the…”
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Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation by Gong, Xiaoyan, Ahner, Annette, Roldan, Ariel, Lukacs, Gergely L., Thibodeau, Patrick H., Frizzell, Raymond A.

“…A newly identified pathway for selective degradation of the common mutant of the cystic fibrosis transmembrane conductance regulator (CFTR), F508del, is…”
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SUMOylation Modulates CFTR Biogenesis: Is the Pathway Druggable? by Ahner, Annette, Frizzell, Raymond A

“…The SUMOylation pathway is involved in the regulation of numerous and diverse cellular functions, nuclear as well as extra-nuclear. Thus, it is not surprising…”
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SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia by Bertrand, Carol A, Zhang, Ruilin, Pilewski, Joseph M, Frizzell, Raymond A

“…Human bronchial epithelial (HBE) cells exhibit constitutive anion secretion that is absent in cells from cystic fibrosis (CF) patients. The identity of this…”
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Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis by Balch, William E, Hutt, Darren M, Herman, David, Rodrigues, Ana P C, Noel, Sabrina, Pilewski, Joseph M, Matteson, Jeanne, Hoch, Ben, Kellner, Wendy, Kelly, Jeffery W, Schmidt, Andre, Thomas, Philip J, Matsumura, Yoshihiro, Skach, William R, Gentzsch, Martina, Riordan, John R, Sorscher, Eric J, Okiyoneda, Tsukasa, Yates, John R, Lukacs, Gergely L, Frizzell, Raymond A, Manning, Gerard, Gottesfeld, Joel M

“…The trafficking defect associated with the cystic fibrosis disease-linked allele of the CFTR chloride channel is alleviated by inhibitors of histone…”
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SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells by Kumar, Parameet, Soni, Dharmendra Kumar, Sen, Chaitali, Larsen, Mads B., Mazan-Mamczarz, Krystyna, Piao, Yulan, De, Supriyo, Gorospe, Myriam, Frizzell, Raymond A., Biswas, Roopa

“…Cystic fibrosis (CF) occurs as a result of mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) gene, which lead to misfolding,…”
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VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis by Ernst, Wayne L., Shome, Kuntala, Wu, Christine C., Gong, Xiaoyan, Frizzell, Raymond A., Aridor, Meir

“…Unesterified cholesterol accumulates in late endosomes in cells expressing the misfolded cystic fibrosis transmembrane conductance regulator (CFTR). CFTR…”
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Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy by Hutt, Darren M., Mishra, Sanjay Kumar, Roth, Daniela Martino, Larsen, Mads Breum, Angles, Frédéric, Frizzell, Raymond A., Balch, William E.

“…The protein chaperones heat shock protein 70 (Hsp70) and Hsp90 are required for de novo folding of proteins and protect against misfolding-related cellular…”
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CK19 stabilizes CFTR at the cell surface by limiting its endocytic pathway degradation by Hou, Xia, Wu, Qingtian, Rajagopalan, Carthic, Zhang, Chunbing, Bouhamdan, Mohamad, Wei, Hongguang, Chen, Xuequn, Zaman, Khalequz, Li, Chunying, Sun, Xiaonan, Chen, Song, Frizzell, Raymond A., Sun, Fei

“…Protein interactions that stabilize the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) at the apical membranes of epithelial cells have not…”
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