Investigating Alternative Transport of Integral Plasma Membrane Proteins from the ER to the Golgi: Lessons from the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Secretory traffic became a topical field because many important cell regulators are plasma membrane proteins (transporters, channels, receptors), being thus key targets in biomedicine and drug discovery. Cystic fibrosis (CF), caused by defects in a single gene encoding the CF transmembrane conductance regulator (CFTR), constitutes the most common of rare diseases and certainly a paradigmatic one.Here we focus on five different approaches that allow biochemical and cellular characterization of CFTR from its co-translational insertion into the ER membrane to its delivery to the plasma membrane.
Faculty of Sciences, BioISI - Biosystems & Integrative Sciences Institute, University of Lisboa, Campo Grande, 1749-016, Lisbon, Portugal. mdamaral@fc.ul.pt.
Margarida D
MD
Amaral
Faculty of Sciences, BioISI - Biosystems & Integrative Sciences Institute, University of Lisboa, Campo Grande, 1749-016, Lisbon, Portugal.
Carlos M
CM
Farinha
Faculty of Sciences, BioISI - Biosystems & Integrative Sciences Institute, University of Lisboa, Campo Grande, 1749-016, Lisbon, Portugal.|Department of Human Genetics, National Health Institute 'Dr. Ricardo Jorge', Av. Padre Cruz, 1649-016, Lisbon, Portugal.
Paulo
P
Matos
Faculty of Sciences, BioISI - Biosystems & Integrative Sciences Institute, University of Lisboa, Campo Grande, 1749-016, Lisbon, Portugal.
Hugo M
HM
Botelho
D018528:ATP-Binding Cassette Transporters / Q000378:metabolism; D015153:Blotting, Western; D003550:Cystic Fibrosis / Q000378:metabolism; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D004705:Endocytosis; D004721:Endoplasmic Reticulum / Q000378:metabolism*; D006026:Glycoside Hydrolases / Q000378:metabolism; D006031:Glycosylation; D006056:Golgi Apparatus / Q000378:metabolism*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008565:Membrane Proteins / Q000378:metabolism*; D008856:Microscopy, Fluorescence; D021381:Protein Transport
D018528:ATP-Binding Cassette Transporters; D008565:Membrane Proteins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D006026:Glycoside Hydrolases
ABC transporters; Biochemistry; CFTR; Cystic fibrosis; Endoplasmic reticulum; Golgi; High-throughput microscopy; N-glycosylation; Plasma membrane; Secretory traffic