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Literature DB >> 7686820

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.

M J Welsh¹, A E Smith.

Abstract

Entities: Chemical Disease Gene Mutation

Mesh：

Substances：

Year: 1993 PMID： 7686820 DOI： 10.1016/0092-8674(93)90353-r

Source DB: PubMed Journal: Cell ISSN： 0092-8674 Impact factor: 41.582

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352 in total

Review 1. Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis.

Authors: Marina S Gelman; Ron R Kopito
Journal: J Clin Invest Date: 2002-12 Impact factor: 14.808

2. Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator.

Authors: X Xiong; A Bragin; J H Widdicombe; J Cohn; W R Skach
Journal: J Clin Invest Date: 1997-09-01 Impact factor: 14.808

Review 3. Pharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptors.

Authors: Alfredo Ulloa-Aguirre; P Michael Conn
Journal: Recent Pat Endocr Metab Immune Drug Discov Date: 2011-01

4. 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

5. Plasma membrane CFTR regulates RANTES expression via its C-terminal PDZ-interacting motif.

Authors: Kim Estell; Gavin Braunstein; Torry Tucker; Karoly Varga; James F Collawn; Lisa M Schwiebert
Journal: Mol Cell Biol Date: 2003-01 Impact factor: 4.272

Review 6. Congenital bilateral absence of the vas deferens as an atypical form of cystic fibrosis: reproductive implications and genetic counseling.

Authors: D A S de Souza; F R Faucz; L Pereira-Ferrari; V S Sotomaior; S Raskin
Journal: Andrology Date: 2017-12-07 Impact factor: 3.842

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.

Review 1. Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis.

2. Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator.

Review 3. Pharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptors.

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

5. Plasma membrane CFTR regulates RANTES expression via its C-terminal PDZ-interacting motif.

Review 6. Congenital bilateral absence of the vas deferens as an atypical form of cystic fibrosis: reproductive implications and genetic counseling.

Review 7. New insights into the pathogenesis of cystic fibrosis sinusitis.

8. Mutations in the linker domain of NBD2 of SUR inhibit transduction but not nucleotide binding.

9. Intracellular mislocalization of mutant podocin and correction by chemical chaperones.

Review 10. Ion Channel Modulators in Cystic Fibrosis.