PXD044975

PXD044975 is an original dataset announced via ProteomeXchange.

Title	Relative quantification of proteins in wild-type vs NDUFS4 -/- in mouse kidney mitochondrial membranes
Description	Respiratory complex I (NADH:ubiquinone oxidoreductase) is essential for cellular energy production and NAD+ homeostasis. Complex I mutations cause neuromuscular, mitochondrial diseases, such as Leigh Syndrome, but their molecular-level consequences remain poorly understood. Here, we use a popular complex I-linked mitochondrial disease model, the ndufs4-/- mouse, to define the structural, biochemical and functional consequences of the absence of subunit NDUFS4. Cryo-EM analyses of mouse-heart ndufs4-/- complex I revealed a loose association of the NADH-dehydrogenase module, and discrete classes containing either assembly factor NDUFAF2 or subunit NDUFS6. Subunit NDUFA12 (that replaces its paralogue NDUFAF2 in mature complex I) is absent from all classes, compounding the deletion of NDUFS4 and preventing maturation of an NDUFS4-free but otherwise complete enzyme. We propose NDUFAF2 as the major recruiter of the NADH-dehydrogenase module during assembly of the complex. Our results provide new molecular-level understanding of the ndufs4-/- mouse model and complex I-linked mitochondrial disease.
HostingRepository	PRIDE
AnnounceDate	2024-01-02
AnnouncementXML	Submission_2024-01-02_12:22:10.054.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Hannah Bridges
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	monohydroxylated residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2023-08-30 08:49:06	ID requested
⏵ 1	2024-01-02 12:22:10	announced

10.1038/S44318-023-00001-4;

submitter keyword: Mitochondria,Mouse, Kidney, NDUFS4 knockout, NDUFS4

Judy Hirst
contact affiliation	MRC Mitochondrial Biology Unit, University of Cambridge
contact email	jh480@cam.ac.uk
lab head
Hannah Bridges
contact affiliation	Mitochondrial Biology Unit
contact email	hrb@mrc-mbu.cam.ac.uk
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD044975
     1. Label: PRIDE project
     2. Name: Relative quantification of proteins in wild-type vs NDUFS4 -/- in mouse kidney mitochondrial membranes