PXD047950-1

PXD047950 is an original dataset announced via ProteomeXchange.

Title	Definition of the composition of the mitochondrial nucleoid in mice
Description	Mitochondrial DNA (mtDNA) is compacted into dynamic structures called mitochondrial nucleoids (mt-nucleoids), with the mitochondrial transcription factor A (TFAM) as the core packaging protein. We generated Tfam-FLAGBAC mice to investigate the mt-nucleoid composition in vivo. Importantly, we showed that the TFAM-FLAG protein is functional and complements the absence of the wild-type TFAM. We identified proteins as core mt-nucleoid components by proteomics analysis of Tfam-FLAGBAC mice. Surprisingly, the mitoribosome subunit MRPS38 was part of the mt-nucleoid, and its downregulation led to the increase of mtDNA replication and transcription. Thus, MRPS38 might have a novel function repressing mtDNA expression. Also, we identified proteins that may stabilize TFAM-FLAG upon depletion of the mitochondrial RNA polymerase despite the absence of mtDNA and induction of the LONP1 protease. Finally, we evaluated the changes in mt-nucleoids caused by very high levels of TFAM-FLAG and discovered eight new interactors that could counteract the high TFAM levels to maintain active mtDNA transcription. Altogether, we demonstrate that the Tfam-FLAGBAC mice are a valuable tool for investigating the mt-nucleoid composition in vivo.
HostingRepository	PRIDE
AnnounceDate	2025-09-24
AnnouncementXML	Submission_2025-09-23_23:05:22.214.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Fredrik Levander
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2023-12-19 07:25:34	ID requested
⏵ 1	2025-09-23 23:05:22	announced

10.1016/J.BBAMCR.2025.119955;

submitter keyword: mouse, mitochondria

Nils-Göran Larsson
contact affiliation	Division of Molecular Metabolism, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden
contact email	nils-goran.larson@ki.se
lab head
Fredrik Levander
contact affiliation	Lund University, Department of Immunotechnology
contact email	fredrik.levander@immun.lth.se
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD047950
     1. Label: PRIDE project
     2. Name: Definition of the composition of the mitochondrial nucleoid in mice