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38 in total

1. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.

Authors: S H Cheng; R J Gregory; J Marshall; S Paul; D W Souza; G A White; C R O'Riordan; A E Smith
Journal: Cell Date: 1990-11-16 Impact factor: 41.582

2. Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome.

Authors: M A Loo; T J Jensen; L Cui; Y Hou; X B Chang; J R Riordan
Journal: EMBO J Date: 1998-12-01 Impact factor: 11.598

3. Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.

Authors: M L Drumm; D J Wilkinson; L S Smit; R T Worrell; T V Strong; R A Frizzell; D C Dawson; F S Collins
Journal: Science Date: 1991-12-20 Impact factor: 47.728

4. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.

Authors: G M Denning; M P Anderson; J F Amara; J Marshall; A E Smith; M J Welsh
Journal: Nature Date: 1992-08-27 Impact factor: 49.962

5. The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.

Authors: Y Yang; S Janich; J A Cohn; J M Wilson
Journal: Proc Natl Acad Sci U S A Date: 1993-10-15 Impact factor: 11.205

6. Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland.

Authors: N Kartner; O Augustinas; T J Jensen; A L Naismith; J R Riordan
Journal: Nat Genet Date: 1992-08 Impact factor: 38.330

7. The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.

Authors: P J Thomas; P Shenbagamurthi; J Sondek; J M Hullihen; P L Pedersen
Journal: J Biol Chem Date: 1992-03-25 Impact factor: 5.157

8. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.

Authors: S Pind; J R Riordan; D B Williams
Journal: J Biol Chem Date: 1994-04-29 Impact factor: 5.157

9. Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel.

Authors: L S Prince; R B Workman; R B Marchase
Journal: Proc Natl Acad Sci U S A Date: 1994-05-24 Impact factor: 11.205

10. Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.

Authors: C L Ward; R R Kopito
Journal: J Biol Chem Date: 1994-10-14 Impact factor: 5.157

34 in total

1. A novel CFTR disease-associated mutation causes addition of an extra N-linked oligosaccharide.

Authors: M M Hämmerle; A A Aleksandrov; X B Chang; J R Riordan
Journal: Glycoconj J Date: 2000-11 Impact factor: 2.916

Review 2. From the ER to the golgi: insights from the study of combined factors V and VIII deficiency.

Authors: W C Nichols; D Ginsburg
Journal: Am J Hum Genet Date: 1999-06 Impact factor: 11.025

3. Comparative genomic sequence analysis of the human and mouse cystic fibrosis transmembrane conductance regulator genes.

Authors: R E Ellsworth; D C Jamison; J W Touchman; S L Chissoe; V V Braden Maduro; G G Bouffard; N L Dietrich; S M Beckstrom-Sternberg; L M Iyer; L A Weintraub; M Cotton; L Courtney; J Edwards; R Maupin; P Ozersky; T Rohlfing; P Wohldmann; T Miner; K Kemp; J Kramer; I Korf; K Pepin; L Antonacci-Fulton; R S Fulton; P Minx; L W Hillier; R K Wilson; R H Waterston; W Miller; E D Green
Journal: Proc Natl Acad Sci U S A Date: 2000-02-01 Impact factor: 11.205

Review 4. Protein misfolding, aggregation, and degradation in disease.

Authors: Niels Gregersen; Lars Bolund; Peter Bross
Journal: Mol Biotechnol Date: 2005-10 Impact factor: 2.695

5. Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures.

Authors: Deborah M Cholon; Wanda K O'Neal; Scott H Randell; John R Riordan; Martina Gentzsch
Journal: Am J Physiol Lung Cell Mol Physiol Date: 2009-12-11 Impact factor: 5.464

Review 6. Chemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosis.

Authors: Susan L Lindquist; Jeffery W Kelly
Journal: Cold Spring Harb Perspect Biol Date: 2011-12-01 Impact factor: 10.005

7. Subcellular localisation, secretion, and post-translational processing of normal cochlin, and of mutants causing the sensorineural deafness and vestibular disorder, DFNA9.

Authors: N G Robertson; S A Hamaker; V Patriub; J C Aster; C C Morton
Journal: J Med Genet Date: 2003-07 Impact factor: 6.318

8. Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator.

Authors: Martina Gentzsch; Xiu-Bao Chang; Liying Cui; Yufeng Wu; Victor V Ozols; Amit Choudhury; Richard E Pagano; John R Riordan
Journal: Mol Biol Cell Date: 2004-04-09 Impact factor: 4.138

9. Amyloid formation in human IAPP transgenic mouse islets and pancreas, and human pancreas, is not associated with endoplasmic reticulum stress.

Authors: R L Hull; S Zraika; J Udayasankar; K Aston-Mourney; S L Subramanian; S E Kahn
Journal: Diabetologia Date: 2009-04-08 Impact factor: 10.122

10. Molecular models of the open and closed states of the whole human CFTR protein.

Authors: Jean-Paul Mornon; Pierre Lehn; Isabelle Callebaut
Journal: Cell Mol Life Sci Date: 2009-08-26 Impact factor: 9.261