Proteomics

Dataset Information

0

CISD3 is required for Complex I function, mitochondrial integrity, and skeletal muscle maintenance


ABSTRACT: 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.

INSTRUMENT(S): timsTOF Pro

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Muscle

SUBMITTER: Thao Thi Nguyen  

LAB HEAD: Ron Mittler

PROVIDER: PXD042593 | Pride | 2024-05-07

REPOSITORIES: Pride

Dataset's files

Source:
Action DRS
20230519_160345_MaleKOvsWT_2techRepeat_Analysis.sne Other
MitoextraKO1_1137.d.zip Other
MitoextraKO1_1137.pepXML Pepxml
MitoextraWT2_1138.d.zip Other
MitoextraWT2_1138.pepXML Pepxml
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