PXD042593-1

PXD042593 is an original dataset announced via ProteomeXchange.

Title	CISD3 is required for Complex I function, mitochondrial integrity, and skeletal muscle maintenance
Description	Mitochondria play a central role in muscle metabolism and function. In skeletal muscles, a unique family of iron-sulfur proteins, termed CISD proteins, support mitochondrial function. The level of these proteins declines with age leading to muscle degeneration. Although the function of the outer mitochondrial proteins CISD1 and CISD2 was defined, the role of the inner mitochondrial protein CISD3, is currently unknown. Here we show that CISD3 deficiency in mice results in muscle atrophy that shares proteomic features with Duchenne Muscular Dystrophy. We further reveal that CISD3 deficiency impairs the function and structure of skeletal muscle mitochondria, and that CISD3 interacts with, and donates its clusters to, Complex I respiratory chain subunit NDUFV2. These findings reveal that CISD3 is important for supporting the biogenesis and function of Complex I, essential for muscle maintenance and function. Interventions that target CISD3 could therefore impact muscle degeneration syndromes, aging, and related conditions.
HostingRepository	PRIDE
AnnounceDate	2024-05-07
AnnouncementXML	Submission_2024-05-07_08:53:24.126.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Thao Thi Nguyen
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	timsTOF Pro

Revision	Datetime	Status	ChangeLog Entry
0	2023-05-31 14:50:06	ID requested
⏵ 1	2024-05-07 08:53:24	announced

10.1073/PNAS.2405123121;

submitter keyword: NEET, DIA-PASEF,CISD, NDUV2

Ron Mittler
contact affiliation	University of Missouri
contact email	mittlerr@missouri.edu
lab head
Thao Thi Nguyen
contact affiliation	University of Missouri-Columbia
contact email	tnguyen24@wisc.edu
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD042593
     1. Label: PRIDE project
     2. Name: CISD3 is required for Complex I function, mitochondrial integrity, and skeletal muscle maintenance