Global Health, Biomedical Research, Novartis, Emeryville, CA, USA.
Biomedical Research, Novartis, Emeryville, CA, USA.
Nat Microbiol. 2024 Nov;9(11):2817-2835. doi: 10.1038/s41564-024-01810-x. Epub 2024 Oct 8.
Diarrhoeal disease caused by Cryptosporidium is a major cause of morbidity and mortality in young and malnourished children from low- and middle-income countries, with no vaccine or effective treatment. Here we describe the discovery of EDI048, a Cryptosporidium PI(4)K inhibitor, designed to be active at the infection site in the gastrointestinal tract and undergo rapid metabolism in the liver. By using mutational analysis and crystal structure, we show that EDI048 binds to highly conserved amino acid residues in the ATP-binding site. EDI048 is orally efficacious in an immunocompromised mouse model despite negligible circulating concentrations, thus demonstrating that gastrointestinal exposure is necessary and sufficient for efficacy. In neonatal calves, a clinical model of cryptosporidiosis, EDI048 treatment resulted in rapid resolution of diarrhoea and significant reduction in faecal oocyst shedding. Safety and pharmacological studies demonstrated predictable metabolism and low systemic exposure of EDI048, providing a substantial safety margin required for a paediatric indication. EDI048 is a promising clinical candidate for the treatment of life-threatening paediatric cryptosporidiosis.
隐孢子虫引起的腹泻病是中低收入国家的年轻和营养不良儿童发病率和死亡率的主要原因,目前尚无疫苗或有效治疗方法。在这里,我们描述了 EDI048 的发现,这是一种隐孢子虫 PI(4)K 抑制剂,旨在在胃肠道的感染部位发挥作用,并在肝脏中迅速代谢。通过使用突变分析和晶体结构,我们表明 EDI048 结合到 ATP 结合位点中高度保守的氨基酸残基。尽管循环浓度可忽略不计,但 EDI048 在免疫功能低下的小鼠模型中具有口服疗效,这表明胃肠道暴露对于疗效是必要且充分的。在新生犊牛中,隐孢子虫病的临床模型中,EDI048 治疗可迅速缓解腹泻,并显著减少粪便卵囊脱落。安全性和药理学研究表明 EDI048 可预测代谢和低全身暴露,为儿科适应症提供了所需的大量安全裕度。EDI048 是治疗危及生命的小儿隐孢子虫病的有前途的临床候选药物。