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IAA-94 is a small molecule chloride channel blocker that acts as a potent inhibitor of Chloride Intracellular Channel 1 (CLIC1). It is primarily utilized as a research tool to investigate the role of chloride channels in cellular signaling, particularly in the context of oxidative stress and vascular remodeling. In preclinical models of pulmonary hypertension (PH), IAA-94 has been shown to reduce right ventricular systolic pressure (RVSP), attenuate vascular remodeling, and restore mitochondrial function by disrupting the CLIC1/RALY signaling axis. This axis facilitates communication between endothelial cells and smooth muscle cells, promoting the proliferative phenotype characteristic of PH. By inhibiting CLIC1, IAA-94 effectively ameliorates disease features in hemolytic PH models, positioning CLIC1 as a potential therapeutic target.
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