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Complexome profiling of mitochondrial protein complexes to study assembly of complex III and supercomplex stability in patients with defects in UQCRH gene

ABSTRACT: UQCRH, also called the hinge protein, is a small subunit of Ubiquinol—cytochrome-c reductase (complex III). Here we used a complexome analysis of primary and immortalized fibroblasts from patients with a homozygous 2 exon deletion in the UQCRH gene and analyzed complex III assembly and altered supercomplex composition.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture, Fibroblast

SUBMITTER: Ilka Wittig

LAB HEAD: Robert Taylor

PROVIDER: PXD022856 | Pride | 2021-09-20

REPOSITORIES: Pride

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