Partnership Aims to Bring HL001, Targeting IPF Progression, Into Trials

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by Marisa Wexler, MS |

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Ube Industries and HiLung are teaming up to advance HL001, an experimental therapy to prevent disease progression in idiopathic pulmonary fibrosis (IPF).

The companies, both based in Japan, anticipate launching clinical studies of HL001 in people by March 2024.

“This most recent agreement solidifies the collaboration between Ube Industries — a highly established chemistry producer, and HiLung — a respiratory cell & organoid biology pioneer, as we accelerate the development of this novel IPF therapy and strive forward to offer the patients, at the earliest timeframe possible, the solutions they have long sought,” the two stated in a press release.

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IPF is characterized by progressive scarring, or fibrosis, in the lungs, which interferes with their ability to take in oxygen. Fibrosis also causes lung tissue to stiffen, making it harder for the lungs to expand during an inhalation. Tissue scarring in IPF is driven by the activity of a number of biological signaling pathways.

HL001, discovered by Ube, is an antagonist, or blocker, of lysophosphatidic acid receptor-1 (LPA1). The LPA1 protein normally binds to certain fatty molecules (lipids) to help govern a host of cellular processes that are believed to drive the progression of fibrosis in IPF.

IPF patients have abnormally high levels of the LPA1 receptor protein, and mice genetically engineered to lack this protein are resistant to developing induced IPF. Another LPA1 antagonist, Bristol-Myers Squibb’s BMS-986020, was reported to reduce the rate of lung function decline in IPF patients in an early clinical trial.

Ube Industries and HiLung first entered into a joint research agreement around HL001 about one year ago. Under that prior agreement, the companies worked together to assess HL001’s potential effectiveness and mechanism of action.

This early work took advantage of HiLung’s proprietary technology to model IPF at the cellular level, a dynamic fibrosing assay based on human iPSC-based respiratory organoids. This technology makes use of induced pluripotent stem cells, or iPSCs, which are stem cells that are obtained by reverting easily accessible cells, like skin or blood cells, back to a stem cell state.

These iPSCs are then grown into lung cells in organoids, which, as the name implies, are cell models meant to mimic the 3D architecture of cells in a human organ.

“These and other preclinical studies have encouraged us that HL001 can make a great contribution to IPF patients and meet the gaping unmet need still present to this day,” the companies stated.

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