AdAlta‘s investigative therapy AD-114, developed for the treatment of idiopathic pulmonary fibrosis (IPF), are in the spotlight at the inaugural IPF Summit Aug. 21-23 in Boston.

The IPF Summit brings together leading researchers and pharmaceutical companies working to create a cure for the disease.

With just a few therapies on the market to treat IPF, patients still experience disease progression. AdAlta, an Australian biotech, developed AD-114 using their novel i-body technology.

I-bodies are proteins that combine the advantages of small molecules, which denote stability, and antibodies, which have specificity and affinity for their intended target.

AD-114, the human version of an antibody in sharks, works to improve the patient’s condition by targeting the chemokine receptor CXCR4. This receptor is found on cells called fibrocytes, which are present in large numbers in IPF and are thought to contribute to the progression of the disease.

Upon binding of AD-114 to CXCR4, anti-fibrotic and anti-inflammatory pathways become activated. Preclinical mouse studies of IPF have shown that treatment with AD-114 leads to anti-inflammatory and anti-fibrotic activity, which are important characteristics for an IPF drug.

AD-114 was granted orphan drug status by the U.S. Food and Drug Administration (FDA), a designation given to therapies being developed to treat rare diseases with high unmet need. The drug’s sponsor qualifies for certain benefits, such as tax incentives, accelerated regulatory timelines, and seven years of market exclusivity.

AdAlta CEO Sam Cobb was invited to speak at the IPF Summit to explain the mechanism through which the therapy works.

“Being invited to speak alongside world experts at the IPF Summit immensely validates the significance of our lead program. Idiopathic pulmonary fibrosis is a disease with high unmet clinical need and we believe AD-114 has the potential to provide a much needed new treatment option,” Cobb said in a press release.

AdAlta is also testing AD-114 in other fibrotic diseases.

“Although our focus with AD-114 is IPF, AD-114 has the potential to treat a wide range of fibrotic conditions, including wet age-related macular degeneration, of which there are 21,000 new cases diagnosed in Australia each year, and non-alcoholic fatty liver disease, which affects around 5.5 million Australians and is a precursor to nonalcoholic steatohepatitis (NASH),” Cobb added.

A Phase 1 clinical trial with AD-114 is expected to begin in 2018.