NDLI: Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs

Content Provider	PubMed Central
Author	Moorsel, Coline H. M. Van Hoffman, Thijs W. Batenburg, Aernoud A. Van Klay, Dymph Vis, Joanne J. Van Der Grutters, Jan C.
Copyright Year	2015
Abstract	Pulmonary fibrosis is the main cause of severe morbidity and mortality in idiopathic interstitial pneumonias (IIP). In the past years, there has been major progress in the discovery of genetic factors that contribute to disease. Genes with highly penetrant mutations or strongly predisposing common risk alleles have been identified in familial and sporadic IIP. This review summarizes genes harbouring causative rare mutations and replicated common predisposing alleles. To date, rare mutations in nine different genes and five risk alleles fulfil this criterion. Mutated genes represent three genes involved in surfactant homeostasis and six genes involved in telomere maintenance. We summarize gene function, gene expressing cells, and pathological consequences of genetic alterations associated with disease. Consequences of the genetic alteration include dysfunctional surfactant processing, ER stress, immune dysregulation, and maintenance of telomere length. Biological evidence shows that these processes point towards a central role for alveolar epithelial type II cell dysfunction. However, tabulation also shows that function and consequence of most common risk alleles are not known. Most importantly, the predisposition of the MUC5B risk allele to disease is not understood. We propose a mechanism whereby MUC5B decreases surface tension lowering capacity of alveolar surfactant at areas with maximal mechanical stress.
Related Links	http://dx.doi.org/10.1155/2015/304186
Starting Page	304186
File Format	PDF
ISSN	23146141
e-ISSN	23146141
Journal	BioMed Research International
Volume Number	2015
Language	English
Publisher	Hindawi Publishing Corporation
Publisher Date	2015-01-01
Access Restriction	Open
Rights Holder	Hindawi Publishing Corporation
Subject Keyword	Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Immunology and Microbiology Medicine Biochemistry, Genetics and Molecular Biology

Sl.	Authority	Responsibilities	Communication Details
1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
5	Website/Portal (Helpdesk)	Queries regarding NDLI and its services	support@ndl.gov.in
6	Contents and Copyright Issues	Queries related to content curation and copyright issues	content@ndl.gov.in
7	National Digital Library of India Club (NDLI Club)	Queries related to NDLI Club formation, support, user awareness program, seminar/symposium, collaboration, social media, promotion, and outreach	clubsupport@ndl.gov.in
8	Digital Preservation Centre (DPC)	Assistance with digitizing and archiving copyright-free printed books	dpc@ndl.gov.in
9	IDR Setup or Support	Queries related to establishment and support of Institutional Digital Repository (IDR) and IDR workshops	idr@ndl.gov.in

Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs.

Idiopathic desquamative interstitial pneumonia in a child: a case report

A Plasminogen Activator Inhibitor-1 Promoter Polymorphism and Idiopathic Interstitial Pneumonia

Gene Delivery Potential of Biofunctional Carbonate Apatite Nanoparticles in Lungs

Morphological and quantitative assessment of mast cells in rheumatoid arthritis associated non‐specific interstitial pneumonia and usual interstitial pneumonia

Understanding gene-environment interactions in a mouse model of Crohn’s disease

Recent advances in understanding idiopathic pulmonary fibrosis

Natural Antioxidant Betanin Protects Rats from Paraquat-Induced Acute Lung Injury Interstitial Pneumonia

Predictors of Mortality for Nursing Home-Acquired Pneumonia: A Systematic Review

Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs

Similar Documents

Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs.

Idiopathic desquamative interstitial pneumonia in a child: a case report

A Plasminogen Activator Inhibitor-1 Promoter Polymorphism and Idiopathic Interstitial Pneumonia

Gene Delivery Potential of Biofunctional Carbonate Apatite Nanoparticles in Lungs

Morphological and quantitative assessment of mast cells in rheumatoid arthritis associated non‐specific interstitial pneumonia and usual interstitial pneumonia

Understanding gene-environment interactions in a mouse model of Crohn’s disease

Recent advances in understanding idiopathic pulmonary fibrosis

Natural Antioxidant Betanin Protects Rats from Paraquat-Induced Acute Lung Injury Interstitial Pneumonia

Predictors of Mortality for Nursing Home-Acquired Pneumonia: A Systematic Review

Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs