P36. 4-hydroxy(phenyl) retinamide (4HPR) worsens the pathological features of ALS-related TBK1-mutant motor neurons by further disrupting the autophagic flux at the elongation phase

TANK-binding kinase 1 (TBK1) is a protein playing a critical role in the activation of pro-survival pathways, by phosphorylating Akt, and in autophagy, as its phosphorylation and activation of the autophagic receptors p62 and optineurin is fundamental for the correct cargo recruitment at the forming autophagosome. Moreover, mutations in TBK1 gene have been recently linked to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). In our study we aimed to uncover the molecular pathological mechanisms characterizing ALS patients carrying mutations in TBK1 gene.

Source: Clinical Neurophysiology - July 10, 2018 Category: Neuroscience Authors: A. Catanese Source Type: research