PXD062725-1

PXD062725 is an original dataset announced via ProteomeXchange.

Title	Human iPSC-derived motor neurons with TDP-43 nuclear loss present early ALS pathological phenotypes
Description	Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disorder characterized by the degeneration of motor neurons and presents a critical need for effective treatment. By establishing and characterizing a human-induced pluripotent stem cell (iPSC)-derived motor neuron (iMN) model with dysfunctional TDP-43 nuclear localization signal (ΔNLS), this study investigates the impact of TAR DNA-binding protein 43 (TDP-43) nuclear depletion in ALS. The model successfully recapitulates functional deficits observed in ALS. Proteomic and metabolomic profiling revealed dysregulation in oxidative stress pathways and altered neurotransmitter release, shedding light on the underlying mechanisms of ALS pathophysiology. This work provides a comprehensive understanding of TDP-43 nuclear depletion in the context of ALS pathogenesis.
HostingRepository	PRIDE
AnnounceDate	2026-07-31
AnnouncementXML	Submission_2026-07-31_02:39:42.084.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Tze Khee Chan
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2025-04-08 03:49:50	ID requested
⏵ 1	2026-07-31 02:39:42	announced

Dataset with its publication pending

submitter keyword: ALS, iPSC-derived motor neuron, TDP-43

Aaron Zefrin Fernandis
contact affiliation	Director, Biology-Discovery, Quantitative Biosciences, MSD International GmBH (Singapore Branch), Singapore
contact email	aaron.zefrin.fernandis@msd.com
lab head
Tze Khee Chan
contact affiliation	Quantitative Biosciences, MSD International GmBH (Singapore Branch), Singapore
contact email	tze.khee.chan@msd.com
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2026/07/PXD062725

PRIDE project URI

• PRIDE
  1. PXD062725
     1. Label: PRIDE project
     2. Name: Human iPSC-derived motor neurons with TDP-43 nuclear loss present early ALS pathological phenotypes