TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

Yu, Chien‐Hsiung; Davidson, Sophia; R. Harapas, Cassandra; B. Hilton, James; J. Mlodzianoski, Michael; Laohamonthonkul, Pawat; Louis, Cynthia; Ren Jie Low, Ronnie; Moecking, Jonas; De Nardo, Dominic; R. Balka, Katherine; J. Calleja, Dale; Moghaddas, Fiona; Ni, Erya; McLean, Catriona; L. Samson, André; Tyebji, Shiraz; J. Tonkin, Christopher; R. Bye, Christopher; J. Turner, Bradley; Pépin, Geneviève; P. Gantier, Michael; L. Rogers, Kelly; McArthur, Kate; J. Crouch, Peter; L. Masters, Seth

doi:10.1016/j.cell.2020.09.020

Public

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

Published in Cell • Oct 1, 2020

Authors:

Chien‐Hsiung Yu

Sophia Davidson

Cassandra R. Harapas

Abstract

Cytoplasmic accumulation of TDP-43 is a disease hallmark for many cases of amyotrophic lateral sclerosis (ALS), associated with a neuroinflammatory cytokine profile related to upregulation of nuclear factor κB (NF-κB) and type I interferon (IFN) pathways. Here we show that this inflammation is drive...

Subject

Biology

Downregulation and upregulation

Cell biology

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