PXD032762-1

PXD032762 is an original dataset announced via ProteomeXchange.

Title	Sideroflexin 4 is a Complex I assembly factor that interacts with the MCIA complex and is required for the assembly of the ND2 module
Description	Sideroflexins (SFXNs) comprise a family of five paralogous proteins (SFXN1-5) in metazoan species. SFXN1/2/3 function as serine transporters and are required for efficient mitochondrial one-carbon (1C) metabolism. SFXN4 is evolutionarily divergent and mutations in SFXN4 give rise to mitochondrial disease, pointing to a distinct function of this protein in mitochondrial biology. Using a combination of genome editing, interaction studies, and quantitative proteomics we show that loss of SFXN4 leads to an isolated complex I assembly defect and that SFXN4 interacts with the core components of the mitochondrial complex I intermediate assembly (MCIA) complex. Our findings suggest that SFXN4 is required for the incorporation of the mtDNA-encoded ND6 subunit in the ND2 assembly module of complex I. These findings provide new insights into the fundamental process of complex I assembly and functional insights into a disease-causing gene belonging to the sideroflexin family.
HostingRepository	PRIDE
AnnounceDate	2022-03-23
AnnouncementXML	Submission_2022-03-23_15:13:32.252.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	David Stroud
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2022-03-23 08:12:26	ID requested
⏵ 1	2022-03-23 15:13:32	announced

Dataset with its publication pending

submitter keyword: Human, Mitochondria, SFXN4

David Stroud
contact affiliation	Department of Biochemistry & Pharmacology, University of Melbourne, Australia
contact email	david.stroud@unimelb.edu.au
lab head
David Stroud
contact affiliation	The University of Melbourne
contact email	david.stroud@unimelb.edu.au
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD032762
     1. Label: PRIDE project
     2. Name: Sideroflexin 4 is a Complex I assembly factor that interacts with the MCIA complex and is required for the assembly of the ND2 module