A novel mouse model of cerebral adrenoleukodystrophy highlights NLRP3 activity in lesion pathogenesis.
Ezzat HashemiIsha Narain SrivastavaAlejandro AguirreEzra Tilahan YosephEsha KaushalAvni AwaniJae Kyu RyuKaterina AkassoglouShahrzad TalebianPauline ChuLaura PisaniPatricia L MusolinoLawrence SteinmanKristian P DoyleWilliam H RobinsonOrr SharpeRomain CayrolPaul J OrchardTroy C LundHannes VogelMax LenailMay Htwe HanJoshua Leith BonkowskyKeith P Van HarenPublished in: bioRxiv : the preprint server for biology (2023)
function predisposes to more severe cerebral inflammation, oxidative stress, fibrin deposition, and Nlrp3 pathway activation, which parallels the findings seen in humans with cALD. We expect this model to enable long-sought investigations into cALD mechanisms and accelerate development of candidate therapies for lesion prevention, cessation, and remyelination.