Proteomics

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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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Publications

Multiple pathways coordinate assembly of human mitochondrial complex IV and stabilization of respiratory supercomplexes.

Lobo-Jarne Teresa T   Pérez-Pérez Rafael R   Fontanesi Flavia F   Timón-Gómez Alba A   Wittig Ilka I   Peñas Ana A   Serrano-Lorenzo Pablo P   García-Consuegra Inés I   Arenas Joaquín J   Martín Miguel A MA   Barrientos Antoni A   Ugalde Cristina C  

The EMBO journal 20200608 14

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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