An aerosolized dual-action autotaxin inhibitor-PPARγ agonist for the treatment of pulmonary fibrosis.

Matralis, Alexios N; Stylianaki, Elli-Anna; Ladopoulou, Eleni M; Kanellopoulou, Paraskevi; Smyrniotis, Stefanos; Magkrioti, Christiana; Papavasileiou, Konstantinos D; Willems, Sabine et al. · Cell Rep Med · 2026

basic_science · Level V

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Abstract

Idiopathic pulmonary fibrosis (IPF) is a fatal fibrotic interstitial lung disease (ILD) with limited therapeutic options. Autotaxin (ATX), an established drug target in IPF, is a secreted lysophospholipase D that catalyzes the extracellular production of lysophosphatidic acid (LPA), a growth-factor-like signaling phospholipid. The many pathologic effects of LPA in the lung include the co-suppression of peroxisome-proliferator-activated receptor γ (PPARγ), a therapeutic target in metabolic disorders. In this report, we introduce EL244, a dual ATX inhibitor and PPARγ agonist endowed with drug-like properties. Developed through repositioning, rational design, targeted synthesis, and pharmacological characterization, EL244 exhibited favorable efficacy and physicochemical profiles. Inhalation of EL244, which alleviates systemic toxicity concerns, attenuated bleomycin (BLM)-induced pulmonary fibrosis and restored respiratory functions; in translation, EL244 attenuated fibrosis in human fibrotic precision-cut lung slices (PCLSs). Therefore, EL244 emerges as a promising clinical candidate for the inhaled treatment of IPF and ILDs.