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Discovering How IPF Drugs Work

July 1, 2017 at 9:53 am

By Paul Schumacker, PhD, editor, American Journal of Respiratory Cell and Molecular Biology

Follow Dr. Schumacker on Twitter @AJRCMB

A Novel Antifibrotic Mechanism of Nintedanib and Pirfenidone: Inhibition of Collagen Fibril Assembly

In 2017, the FDA approved pirfenidone and nintedanib based on clinical trials that showed the drugs slowed the progression of idiopathic pulmonary fibrosis. But the drugs’ mechanisms of action are unclear. In their July American Journal of Respiratory Cell and Molecular Biology article, Larissa Knüppel and colleagues report on their study of fibroblasts from IPF patients and healthy donors treated with the two drugs. They found that “both drugs act on important regulatory levels in collagen synthesis and processing.” Nintedanib, however, more effectively downregulated “profibrotic gene expression and collagen secretion.” The researchers also provide the first evidence that both drugs inhibit collagen I fibril formation. This discovery, they write, demonstrates “a completely novel mechanism of action for both drugs” and may represent a target for development of other IPF drugs.


July Highlights

Genetic Association and Risk Scores in a Chronic Obstructive Pulmonary Disease Meta-Analysis of 16,707 Subjects

Transcriptomic Analysis of Lung Tissue from Cigarette Smoke–Induced Emphysema Murine Models and Human Chronic Obstructive Pulmonary Disease Show Shared and Distinct Pathways

Three Unique Interstitial Macrophages in the Murine Lung at Steady State

Regulation of MUC5B Expression in Idiopathic Pulmonary Fibrosis


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