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Title: CFTR processing, trafficking and interactions

Authors:
; ; ; ORCiD logo; ORCiD logo
Publication Date:
Sponsoring Org.:
USDOE Office of Nuclear Energy (NE), Nuclear Fuel Cycle and Supply Chain
OSTI Identifier:
1703966
Grant/Contract Number:  
UID/MULTI/04046/2013; SFRH/BPD/93017/2013
Resource Type:
Publisher's Accepted Manuscript
Journal Name:
Journal of Cystic Fibrosis
Additional Journal Information:
Journal Name: Journal of Cystic Fibrosis Journal Volume: 19 Journal Issue: S1; Journal ID: ISSN 1569-1993
Publisher:
Elsevier
Country of Publication:
Country unknown/Code not available
Language:
English

Citation Formats

Amaral, Margarida D., Hutt, Darren M., Tomati, Valeria, Botelho, Hugo M., and Pedemonte, Nicoletta. CFTR processing, trafficking and interactions. Country unknown/Code not available: N. p., 2020. Web. https://doi.org/10.1016/j.jcf.2019.10.017.
Amaral, Margarida D., Hutt, Darren M., Tomati, Valeria, Botelho, Hugo M., & Pedemonte, Nicoletta. CFTR processing, trafficking and interactions. Country unknown/Code not available. https://doi.org/10.1016/j.jcf.2019.10.017
Amaral, Margarida D., Hutt, Darren M., Tomati, Valeria, Botelho, Hugo M., and Pedemonte, Nicoletta. Sun . "CFTR processing, trafficking and interactions". Country unknown/Code not available. https://doi.org/10.1016/j.jcf.2019.10.017.
@article{osti_1703966,
title = {CFTR processing, trafficking and interactions},
author = {Amaral, Margarida D. and Hutt, Darren M. and Tomati, Valeria and Botelho, Hugo M. and Pedemonte, Nicoletta},
abstractNote = {},
doi = {10.1016/j.jcf.2019.10.017},
journal = {Journal of Cystic Fibrosis},
number = S1,
volume = 19,
place = {Country unknown/Code not available},
year = {2020},
month = {3}
}

Journal Article:
Free Publicly Available Full Text
Publisher's Version of Record
https://doi.org/10.1016/j.jcf.2019.10.017

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Works referenced in this record:

Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies
journal, July 2014

  • Fanen, Pascale; Wohlhuter-Haddad, Adeline; Hinzpeter, Alexandre
  • The International Journal of Biochemistry & Cell Biology, Vol. 52
  • DOI: 10.1016/j.biocel.2014.02.023

Hallmarks of therapeutic management of the cystic fibrosis functional landscape
journal, November 2015


Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene
journal, August 2013

  • Sosnay, Patrick R.; Siklosi, Karen R.; Van Goor, Fredrick
  • Nature Genetics, Vol. 45, Issue 10
  • DOI: 10.1038/ng.2745

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane
journal, July 2010


A Proteomic Variant Approach (ProVarA) for Personalized Medicine of Inherited and Somatic Disease
journal, September 2018

  • Hutt, Darren M.; Loguercio, Salvatore; Campos, Alexandre Rosa
  • Journal of Molecular Biology, Vol. 430, Issue 18
  • DOI: 10.1016/j.jmb.2018.06.017

∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis
journal, November 2015

  • Pankow, Sandra; Bamberger, Casimir; Calzolari, Diego
  • Nature, Vol. 528, Issue 7583
  • DOI: 10.1038/nature15729

Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding of CFTR in Cystic Fibrosis
journal, November 2006


Protein Traffic Disorders: an Effective High-Throughput Fluorescence Microscopy Pipeline for Drug Discovery
journal, March 2015

  • Botelho, Hugo M.; Uliyakina, Inna; Awatade, Nikhil T.
  • Scientific Reports, Vol. 5, Issue 1
  • DOI: 10.1038/srep09038

Genome-wide RNAi screening identifies human proteins with a regulatory function in the early secretory pathway
journal, June 2012

  • Simpson, Jeremy C.; Joggerst, Brigitte; Laketa, Vibor
  • Nature Cell Biology, Vol. 14, Issue 7
  • DOI: 10.1038/ncb2510

Most F508del-CFTR Is Targeted to Degradation at an Early Folding Checkpoint and Independently of Calnexin
journal, June 2005


Revertants, Low Temperature, and Correctors Reveal the Mechanism of F508del-CFTR Rescue by VX-809 and Suggest Multiple Agents for Full Correction
journal, July 2013


Proteomic interaction profiling reveals KIFC1 as a factor involved in early targeting of F508del-CFTR to degradation
journal, July 2018

  • Canato, Sara; Santos, João D.; Carvalho, Ana S.
  • Cellular and Molecular Life Sciences, Vol. 75, Issue 24
  • DOI: 10.1007/s00018-018-2896-7

Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic Reticulum
journal, April 2019


Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists
journal, November 2001

  • Galietta, Luis V. J.; Jayaraman, Sujatha; Verkman, A. S.
  • American Journal of Physiology-Cell Physiology, Vol. 281, Issue 5
  • DOI: 10.1152/ajpcell.2001.281.5.C1734

Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation
journal, July 2015

  • Tomati, Valeria; Sondo, Elvira; Armirotti, Andrea
  • Scientific Reports, Vol. 5, Issue 1
  • DOI: 10.1038/srep12138

Mechanisms for Rescue of Correctable Folding Defects in CFTRΔF508
journal, September 2009

  • Grove, Diane E.; Rosser, Meredith F. N.; Ren, Hong Yu
  • Molecular Biology of the Cell, Vol. 20, Issue 18
  • DOI: 10.1091/mbc.e08-09-0929

Sequential Quality-Control Checkpoints Triage Misfolded Cystic Fibrosis Transmembrane Conductance Regulator
journal, August 2006


Pharmacological Inhibition of the Ubiquitin Ligase RNF5 Rescues F508del-CFTR in Cystic Fibrosis Airway Epithelia
journal, July 2018


High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel
journal, January 2018

  • Tomati, Valeria; Pesce, Emanuela; Caci, Emanuela
  • Journal of Biological Chemistry, Vol. 293, Issue 4
  • DOI: 10.1074/jbc.M117.816595

Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways
journal, July 2013

  • Ahner, Annette; Gong, Xiaoyan; Frizzell, Raymond A.
  • FEBS Journal, Vol. 280, Issue 18
  • DOI: 10.1111/febs.12415

UBXD1 is a VCP-interacting protein that is involved in ER-associated degradation
journal, May 2009

  • Nagahama, Masami; Ohnishi, Machi; Kawate, Yumiko
  • Biochemical and Biophysical Research Communications, Vol. 382, Issue 2
  • DOI: 10.1016/j.bbrc.2009.03.012

Adapting Proteostasis for Disease Intervention
journal, February 2008

  • Balch, William E.; Morimoto, Richard I.; Dillin, Andrew
  • Science, Vol. 319, Issue 5865
  • DOI: 10.1126/science.1141448

Chemical and Biological Approaches Synergize to Ameliorate Protein-Folding Diseases
journal, September 2008


Identification of the cystic fibrosis gene: genetic analysis
journal, September 1989


Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
journal, September 1989


Identification of the cystic fibrosis gene: chromosome walking and jumping
journal, September 1989


Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
journal, October 2009

  • Van Goor, F.; Hadida, S.; Grootenhuis, P. D. J.
  • Proceedings of the National Academy of Sciences, Vol. 106, Issue 44
  • DOI: 10.1073/pnas.0904709106