Autophagy meets fused in sarcoma-positive stress granules.

Abstract:

:Mutations in fused in sarcoma and/or translocated in liposarcoma (FUS, TLS or FUS) are linked to familial cases of amyotrophic lateral sclerosis (ALS). Mutant FUS selectively accumulates into discrete cytosolic structures known as stress granules under various stress conditions. In addition, mutant FUS expression can alter the dynamics and morphology of stress granules. Although the link between mutant FUS and stress granules is well established, the mechanisms modulating stress granule formation and disassembly in the context of ALS are poorly understood. In this issue of Neurobiology of Aging, Ryu et al. uncover the impact of autophagy on the potential toxicity of mutant FUS-positive stress granules. The authors provide evidence indicating that enhanced autophagy activity reduces the number of stress granules, which in the case of cells containing mutant FUS-positive stress granules, is neuroprotective. Overall, this study identifies an intersection between the proteostasis network and alterations in RNA metabolism in ALS through the dynamic assembly and disassembly of stress granules.

journal_name

Neurobiol Aging

journal_title

Neurobiology of aging

authors

Matus S,Bosco DA,Hetz C

doi

10.1016/j.neurobiolaging.2014.08.019

subject

Has Abstract

pub_date

2014-12-01 00:00:00

pages

2832-2835

issue

12

eissn

0197-4580

issn

1558-1497

pii

S0197-4580(14)00532-6

journal_volume

35

pub_type

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