PXD053461-1

PXD053461 is an original dataset announced via ProteomeXchange.

Title	Complexome profiling of mitochondrial protein complexes from CRISPR/Cas9 edited U2OS cells lacking COA5
Description	Mitochondria are complex cellular organelles that harness the process of oxidative phosphorylation (OXPHOS) to provide a usable energy source for the cell and body. The failure of mitochondria to achieve this is associated with a wide spectrum of disorders that affect both children and adults with an incidence of at least 1/4300 1. Due to the dual genetic control of genes encoding the structural components of the mitochondrial OXPHOS system, mitochondrial disorders can occur via almost any pattern of inheritance. The assembly of mitochondrial OXPHOS complexes is an intricate process and requires tight co-regulation of two evolutionarily distinct gene expression machineries. In addition, early- and late-stage ancillary chaperones have essential roles in coordinating OXPHOS assembly, regulating the step-wise insertion of individual subunits and /or essential co-factors. Mitochondrial OXPHOS complex IV (cytochrome c oxidase) comprises of 14 subunits, with all three catalytic subunits (COX1, COX2, and COX3) encoded by mitochondrial DNA. Complex IV represents the final stage of electron transfer across the electron transport chain by cytochrome c to oxygen as the final electron acceptor in OXPHOS. Previous work in our lab and others identified pathogenic variants in a novel complex IV assembly factor COA5 associated with cardiomyopathy and defective complex IV assembly. Here we used complexome profiling to study the role of COA5 in early stage complex IV assembly using a CRISPR/Cas9-mediated COA5 gene knockout cell line.
HostingRepository	PRIDE
AnnounceDate	2025-01-07
AnnouncementXML	Submission_2025-01-07_04:39:03.727.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	iodoacetic acid derivatized residue
Instrument	Q Exactive Plus

Revision	Datetime	Status	ChangeLog Entry
0	2024-06-27 14:00:29	ID requested
⏵ 1	2025-01-07 04:39:04	announced

10.26508/LSA.202403013;

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

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

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PRIDE project URI

• PRIDE
  1. PXD053461
     1. Label: PRIDE project
     2. Name: Complexome profiling of mitochondrial protein complexes from CRISPR/Cas9 edited U2OS cells lacking COA5