PXD055511

PXD055511 is an original dataset announced via ProteomeXchange.

Title	RTN4IP1 is essential for the final stages of mitochondrial complex I assembly
Description	A biochemical deficiency of mitochondrial complex I (CI) underlies ~30% of cases of primary mitochondrial disease, yet the inventory of molecular machinery required for CI assembly remains incomplete. We previously characterised patients with isolated CI deficiency caused by segregating variants in RTN4IP1, encoding a poorly characterised mitochondrial protein. Here, we used MS-complexome profiling to characterise the functional consequences of RTN4IP1 deficiency in both patient-derived fibroblasts and U2OS-knockout cells, demonstrating that RTN4IP1 is a bona fide CI assembly factor.
HostingRepository	PRIDE
AnnounceDate	2025-08-08
AnnouncementXML	Submission_2025-08-08_03:56:30.616.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Ilka Wittig
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2024-09-03 04:25:09	ID requested
⏵ 1	2025-08-08 03:56:31	announced

Dataset with its publication pending

submitter keyword: oxidative phosphorylation system,LC-MSMS, complexome profiling, complex I assembly

Robert Taylor
contact affiliation	Mitochondrial Research Group, Faculty of Medical Sciences, Newcastle University, UK
contact email	robert.taylor@ncl.ac.uk
lab head
Ilka Wittig
contact affiliation	Functional Proteomics, Goethe University, Frankfurt am Main , Germany
contact email	wittig@med.uni-frankfurt.de
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/2025/08/PXD055511

PRIDE project URI

• PRIDE
  1. PXD055511
     1. Label: PRIDE project
     2. Name: RTN4IP1 is essential for the final stages of mitochondrial complex I assembly