Research ArticleIdiopathic Pulmonary Fibrosis

Chitinase 3–Like 1 Suppresses Injury and Promotes Fibroproliferative Responses in Mammalian Lung Fibrosis

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Science Translational Medicine  11 Jun 2014:
Vol. 6, Issue 240, pp. 240ra76
DOI: 10.1126/scitranslmed.3007096

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Two-Face Chitinase

Idiopathic pulmonary fibrosis is a devastating—currently incurable—disease where scars develop deep in the lung, making it hard to breathe. Now, Zhou et al. report a breath of fresh air for IPF. They found that the protein chitinase 3–like 1 (CHI3L1) is elevated in IPF patients, and that high levels of CHI3L1 are associated with disease progression. However, things were not as simple as they seemed. CHI3L1 actually played a protective, anti-apoptotic role against lung injury, but contributed to fibrosis during the repair phase, in part through inducing myofibroblast transformation. This insight into the two sides of CHI3L1 provides mechanistic understanding of the pathogenesis of IPF, which is necessary to the development of successful therapeutics.