PXD061618-1

PXD061618 is an original dataset announced via ProteomeXchange.

Title	COX6B1 secures a redox-sensitive step in early cytochrome c oxidase assembly
Description	COX6B is one of the eleven nuclear-encoded subunits of the human mitochondrial inner-membrane cytochrome c oxidase (cIV), which is located in the intermembrane space-facing region of cIV. In mammals, there are two COX6B isoforms: the ubiquitous isoform COX6B1, expressed in all cell types and tissues, and COX6B2 present only in testes and certain types of cancer. Rare COX6B1 missense pathogenic variants have been identified as the cause of childhood-onset mitochondrial encephalopathy associated with cIV deficiency. Despite the relevance of COX6B1 in mitochondrial physiopathology, its molecular role in cIV biogenesis and/or maintenance had not been thoroughly characterized. In spite of the assigned role for COX6B1 as a late incorporation subunit into an almost complete cIV, a COX6B1 human cell line knock-out (KO) exhibited a total loss of cIV. To get a deeper insight into the mechanisms that drive the lack of assembly or destabilization of cIV in the absence of COX6B1, we also used the COX6B1 KO cell background to express an alternative oxidase (AOX) and COX6B1 carrying the R20C and R20H pathogenic variants. These analyses have led us to conclude that the COX6B1 subunit does not only contribute to the stabilization of cIV in the late assembly stages, but is also indispensable for redox-sensitive early cIV assembly steps. In addition, this study has evidenced the incorporation of partially assembled cIV modules directly into supercomplex structures, supporting the ‘cooperative assembly’ model for respiratory chain biogenesis.
HostingRepository	PRIDE
AnnounceDate	2025-12-15
AnnouncementXML	Submission_2025-12-15_09:08:44.991.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Marek Vrbacky
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606;
ModificationList	iodoacetamide derivatized residue
Instrument	Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2025-03-09 12:10:05	ID requested
⏵ 1	2025-12-15 09:08:45	announced

Dataset with its publication pending

submitter keyword: mitochondria, alternative oxidase (AOX),cytochrome c oxidase, respiratory chain biogenesis, complexome profiling

Petr Pecina
contact affiliation	Laboratory of Bioenergetics Institute of Physiology, Czech Academy of Sciences Prague Czech Republic
contact email	Petr.Pecina@fgu.cas.cz
lab head
Marek Vrbacky
contact affiliation	Czech Academy of Sciences
contact email	proteom.krc@gmail.com
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD061618
     1. Label: PRIDE project
     2. Name: COX6B1 secures a redox-sensitive step in early cytochrome c oxidase assembly