PXD038042

PXD038042 is an original dataset announced via ProteomeXchange.

Title	IFNγ protects motor neurons from oxidative stress via enhanced global protein synthesis in FUS-associated Amyotrophic Lateral Sclerosis
Description	Amyotrophic lateral sclerosis type 6 (ALS6) is a familial subtype of ALS linked to Fused in Sarcoma (FUS) gene mutation. FUS mutations lead to decreased global protein synthesis, but the mechanism that drives this has not been established. Here, we used ALS6 patient-derived induced pluripotent stem cells (hIPSCs) to study the effect of the ALS6 FUSR521H mutation on the translation machinery in motor neurons (MNs). We find, in agreement with findings of others, that protein synthesis is decreased in ALS6 MNs. Furthermore, ALS6 MNs are more sensitive to oxidative stress and display reduced expression of TGF-β and mTORC gene pathways when stressed. Finally, we show that IFNγ treatment reduces apoptosis of ALS6 MNs exposed to oxidative stress and partially restores the translation rates in ALS6 MNs. Overall, these findings suggest that a functional IFNγ response is important for FUS-mediated protein synthesis, possibly by FUS nuclear translocation in ALS6.
HostingRepository	PRIDE
AnnounceDate	2024-01-26
AnnouncementXML	Submission_2024-01-26_06:50:55.511.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD038042
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	AMANDA ASSONI
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2022-11-08 08:02:34	ID requested
⏵ 1	2024-01-26 06:50:56	announced

10.6019/PXD038042;

10.1111/bpa.13206;

Assoni AF, Guerrero EN, Wardenaar R, Oliveira D, Bakker PL, Alves LM, Carvalho VM, Okamoto OK, Zatz M, Foijer F, protects motor neurons from oxidative stress via enhanced global protein synthesis in FUS-associated amyotrophic lateral sclerosis. Brain Pathol, 34(1):e13206(2024) [pubmed]

submitter keyword: ALS

ALS6

FUS

protein synthesis

IFNy

Floris Foijer
contact affiliation	European Research Institute for the Biology of Ageing, University of Groningen, Groningen, 9713 AV, the Netherlands.
contact email	f.foijer@umcg.nl
lab head
AMANDA ASSONI
contact affiliation	University of São Paulo
contact email	amandafassoni@gmail.com
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD038042
     1. Label: PRIDE project
     2. Name: IFNγ protects motor neurons from oxidative stress via enhanced global protein synthesis in FUS-associated Amyotrophic Lateral Sclerosis