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Vous êtes ici : Accueil / Équipes / Mort Cellulaire Régulée et Génétique de la Neurodégénerescence - B. Mollereau / Publications / Is WDR45 the missing link for ER stress-induced autophagy in beta-propeller associated neurodegeneration?

Is WDR45 the missing link for ER stress-induced autophagy in beta-propeller associated neurodegeneration?

Bertrand Mollereau and Ludivine Walter (2019)

Autophagy, 15(12):2163-2164.

Beta-propeller protein-associated neurodegeneration (BPAN) is caused by mutations inthe autophagy gene WDR45/WIPI4. In human, BPAN is associated with staticencephalopathy in childhood and neurodegeneration in adulthood (SENDA). It has beenproposed that WDR45 mutations cause neurodegeneration due to defective autophagy.Whether these mutations cause a global attenuation or a defect in a subset ofautophagy functions is unknown. Based on a recent study showing that wdr45 knockoutmice exhibit defective autophagy associated with an increased ER stress, we proposethat ER-mediated autophagy, a selective activation of autophagy, is defective inmouse and cellular models of BPAN. We discuss the implication of these findings onthe pathophysiological relevance of the relationship between ER stress and autophagyin BPAN as well as other neurodegenerative diseases exhibiting ER stress anddefective autophagy.

 
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