Mechanisms of protein misfolding in conformational lung diseases.

Hdl Handle:
http://hdl.handle.net/10147/264500
Title:
Mechanisms of protein misfolding in conformational lung diseases.
Authors:
McElvaney, N G; Greene, C M
Affiliation:
Respiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland.
Citation:
Mechanisms of protein misfolding in conformational lung diseases. 2012, 12 (7):850-9 Curr. Mol. Med.
Journal:
Current molecular medicine
Issue Date:
Aug-2012
URI:
http://hdl.handle.net/10147/264500
PubMed ID:
22697345
Abstract:
Genetic or environmentally-induced alterations in protein structure interfere with the correct folding, assembly and trafficking of proteins. In the lung the expression of misfolded proteins can induce a variety of pathogenetic effects. Cystic fibrosis (CF) and alpha-1 antitrypsin (AAT) deficiency are two major clinically relevant pulmonary disorders associated with protein misfolding. Both are genetic diseases the primary causes of which are expression of mutant alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) and SERPINA1, respectively. The most common and best studied mutant forms of CFTR and AAT are ΔF508 CFTR and the Glu342Lys mutant of AAT called ZAAT, respectively. Non-genetic mechanisms can also damage protein structure and induce protein misfolding in the lung. Cigarette-smoke contains oxidants and other factors that can modify a protein's structure, and is one of the most significant environmental causes of protein damage within the lung. Herein we describe the mechanisms controlling the folding of wild type and mutant versions of CFTR and AAT proteins, and explore the consequences of cigarette-smoke-induced effects on the protein folding machinery in the lung.
Item Type:
Article
Language:
en
MeSH:
Cystic Fibrosis Transmembrane Conductance Regulator; Endoplasmic Reticulum Stress; Humans; Lung Diseases; Protein Folding
ISSN:
1875-5666

Full metadata record

DC FieldValue Language
dc.contributor.authorMcElvaney, N Gen_GB
dc.contributor.authorGreene, C Men_GB
dc.date.accessioned2013-01-08T15:49:39Z-
dc.date.available2013-01-08T15:49:39Z-
dc.date.issued2012-08-
dc.identifier.citationMechanisms of protein misfolding in conformational lung diseases. 2012, 12 (7):850-9 Curr. Mol. Med.en_GB
dc.identifier.issn1875-5666-
dc.identifier.pmid22697345-
dc.identifier.urihttp://hdl.handle.net/10147/264500-
dc.description.abstractGenetic or environmentally-induced alterations in protein structure interfere with the correct folding, assembly and trafficking of proteins. In the lung the expression of misfolded proteins can induce a variety of pathogenetic effects. Cystic fibrosis (CF) and alpha-1 antitrypsin (AAT) deficiency are two major clinically relevant pulmonary disorders associated with protein misfolding. Both are genetic diseases the primary causes of which are expression of mutant alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) and SERPINA1, respectively. The most common and best studied mutant forms of CFTR and AAT are ΔF508 CFTR and the Glu342Lys mutant of AAT called ZAAT, respectively. Non-genetic mechanisms can also damage protein structure and induce protein misfolding in the lung. Cigarette-smoke contains oxidants and other factors that can modify a protein's structure, and is one of the most significant environmental causes of protein damage within the lung. Herein we describe the mechanisms controlling the folding of wild type and mutant versions of CFTR and AAT proteins, and explore the consequences of cigarette-smoke-induced effects on the protein folding machinery in the lung.en_GB
dc.language.isoenen
dc.rightsArchived with thanks to Current molecular medicineen_GB
dc.subject.meshCystic Fibrosis Transmembrane Conductance Regulator-
dc.subject.meshEndoplasmic Reticulum Stress-
dc.subject.meshHumans-
dc.subject.meshLung Diseases-
dc.subject.meshProtein Folding-
dc.titleMechanisms of protein misfolding in conformational lung diseases.en_GB
dc.typeArticleen
dc.contributor.departmentRespiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland.en_GB
dc.identifier.journalCurrent molecular medicineen_GB
dc.description.provinceLeinsteren
All Items in Lenus, The Irish Health Repository are protected by copyright, with all rights reserved, unless otherwise indicated.