Proteomics

Dataset Information

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Complexome profiling of human cybrids laking mitochondrial genes Cox1 or Cox2

ABSTRACT: Mitochondrial respiratory chain (MRC) complexes I, III and IV are associated into large molecular structures named supercomplexes (SCs) or respirasomes, whose functional roles remain to be fully understood. Biochemical analyses in a diversity of OXPHOS-deficient cellular and animal models support the idea that one of the SCs function is to confer stability to individual MRC complexes, in particular to complex I (CI). To gain insight into the mechanisms that regulate the structural interdependences among MRC complexes, we performed complexome profiling of human cybrids laking mitochondrial genes Cox1 or Cox2 and compared protein assemblies and intermediates to control 143B cells.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Ilka Wittig  

LAB HEAD: Cristina Ugalde

PROVIDER: PXD017840 | Pride | 2020-07-01

REPOSITORIES: Pride

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BNE_gel.jpg Other
Complexomedata.xlsx Xlsx
P18_057_Ugalde_143B_01.raw Raw
P18_057_Ugalde_143B_02.raw Raw
P18_057_Ugalde_143B_03.raw Raw
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Publications

Mitochondrial respiratory chain complexes I, III, and IV can associate into larger structures termed supercomplexes or respirasomes, thereby generating structural interdependences among the individual complexes yet to be understood. In patients, nonsense mutations in complex IV subunit genes cause severe encephalomyopathies randomly associated with pleiotropic complex I defects. Using complexome profiling and biochemical analyses, we have explored the structural rearrangements of the respiratory  ...[more]

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