Cargando…
Cystic fibrosis – a multiorgan protein misfolding disease
Cystic fibrosis (CF) is a heterogeneous multiorgan disease caused by mutations in the CFTR gene leading to misfolding (and other defects) and consequent dysfunction of CFTR protein. The majority of mutations cause a severe CF phenotype, and people with this condition will require a wide variety of m...
Autores principales: | Fraser-Pitt, Douglas, O’Neil, Deborah |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Future Science Ltd
2015
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5137970/ https://www.ncbi.nlm.nih.gov/pubmed/28031875 http://dx.doi.org/10.4155/fso.15.57 |
Ejemplares similares
-
Activity of Cysteamine against the Cystic Fibrosis Pathogen Burkholderia cepacia Complex
por: Fraser-Pitt, Douglas, et al.
Publicado: (2016) -
Cellular stress responses in protein misfolding diseases
por: Duennwald, Martin L
Publicado: (2015) -
Unraveling protein misfolding diseases using model systems
por: Peffer, Sara, et al.
Publicado: (2015) -
Cysteamine (Lynovex®), a novel mucoactive antimicrobial & antibiofilm agent for the treatment of cystic fibrosis
por: Charrier, Cedric, et al.
Publicado: (2014) -
An Open-Label Investigation of the Pharmacokinetics and Tolerability of Oral Cysteamine in Adults with Cystic Fibrosis
por: Devereux, Graham, et al.
Publicado: (2016)