Potential drug target discovered for idiopathic pulmonary fibrosis

By Oliver Worsley

Dr. Claudia Staab-Weijnitz and Prof. Oliver Eickelberg

Oliver Eickelberg and Dr. Claudia Staab-Weijnitz from the Helmholtz Zentrum Munchen have shown with help from their collaborators at Yale University that levels of a protein called FK506-binding protein 10 (FKBP10) are elevated in patients with idiopathic pulmonary fibrosis (IPF).

IPF is a chronic disease affecting the connective tissue between air sacs in the lungs and has an extremely poor outcome--on average, patients have a life expectancy of around two to three years after diagnosis. IPF is characterized by fibrosis, the formation of scar tissue that builds up in the lungs and progressively impairs lung function through decreasing lung elasticity.

Using microarray studies to probe their clinical IPF samples for relative gene expression, they found that the gene FKBP10 was significantly increased in its expression and importantly, FKBP10 was similarly elevated at the protein level. The FKBP10 protein is known to synthesize and secrete collagen, a major component of scar tissue.

By experimentally knocking down FKBP10 in vitro they could reduce the collagen secretion in a cell line. This work puts forward the possibility that inhibiting FKBP10 may reduce the amount of scar tissue in IPF patients, as well as proposing a mechanism for a previously described drug which shows antifibrotic behavior by targeting FKBP10.

Eickelberg pins his hopes on this novel target and is leading an effort in his lab to further understand the pathogenesis of IPF. He admits collaborative efforts are needed: "My foremost objective is to help develop an effective treatment that will completely halt the progression of IPF in the patient. … These approaches are best developed in international networks."

Staab-Weijnitz sees the work having broad implications. "FKBP10 represents a potential new target molecule for the individualized therapy of IPF. … In the future, these results could also lead to new therapeutic options for the treatment of other fibrotic diseases."

- here's the release

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