Idiopathic Pulmonary Fibrosis: A Genetic Disease That Involves Mucociliary Dysfunction of the Peripheral Airways

Idiopathic pulmonary fibrosis (IPF) is an incurable complex genetic disorder that is associated with sequence changes in 7 genes (MUC5B, TERT, TERC, RTEL1, PARN, SFTPC, and SFTPA2) and with variants in at least 11 novel loci. We have previously found that 1) a common gain-of-function promoter varian...

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Gepubliceerd in:Physiol Rev
Hoofdauteurs: Evans, Christopher M., Fingerlin, Tasha E., Schwarz, Marvin I., Lynch, David, Kurche, Jonathan, Warg, Laura, Yang, Ivana V., Schwartz, David A.
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: American Physiological Society 2016
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Online toegang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5243224/
https://ncbi.nlm.nih.gov/pubmed/27630174
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/physrev.00004.2016
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