Discusses the relationship between the CFTR protein and cystic fibrosis Explains how the protein structure and structure-defects affect the function of CFTR

Presents CFTR-based and independent therapies to correct the basic defect

Discusses the relationship between the CFTR protein and cystic fibrosis Explains how the protein structure and structure-defects affect the function of CFTR Presents CFTR-based and independent therapies to correct the basic defect

Autorentext

Carlos M Farinha obtained his Ph.D. (2003) and Habilitation (2015) in Biochemistry from the University of Lisbon. He has worked at the National Institute of Health Dr. Ricardo Jorge and at the Faculty of Sciences, University of Lisbon with training periods at the Johns Hopkins University School of Medicine, Baltimore, MD, USA and at the University of Pittsburgh School of Medicine Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. Currently, he is Assistant Professor with habilitation at the Faculty of Sciences, University of Lisbon, and Principal Investigator at BioISI Biosystems and Integrative Sciences Institute. His research focuses on the molecular mechanisms of cystic fibrosis (CF), in particular the endoplasmic reticulum retention and early degradation of CFTR's most common mutant and characterization of the role of CFTR interacting proteins. He received the 2012 ERS Romain Pauwels Research Award from the European Respiratory Society for research into the different aspects of CFTR biology.

Inhalt
Cystic fibrosis: an overview.- CFTR gene and protein.- CFTR in the cell.- CFTR function.- Therapies aimed at correcting the basic defect.