HL001 / HiLung, Ube Corp - LARVOL DELTA

Preclinical evaluation of HL001, a novel lysophosphatidic acid 1 (LPA1) receptor antagonist, for idiopathic pulmonary fibrosis utilizing physiologically relevant stem cell-derived lung organoids (ERS 2023) - "Fibrotic phenotypes were induced by bleomycin followed by treatment with HL001...Results Fibrotic phenotypes in organoid including 'tissue shrinking' were ameliorated by HL001 compared to nintedanib...Conclusions Human stem cell-derived lung organoid models were highly useful for preclinical evaluation of drug effects. HL001 revealed significant anti-fibrotic effects both in murine models and human organoid models, which indicates high potential of HL001 as a candidate for idiopathic pulmonary fibrosis.; Cell and molecular biology; Physiology"

Preclinical • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Orphan Designation: Treatment of idiopathic pulmonary fibrosis (FDA) - Date Designated: 06/12/2023

Orphan drug • Idiopathic Pulmonary Fibrosis • Respiratory Diseases