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A MITOCHONDRIAL STRESS RESPONSE TRIGGERED BY DEFECTS IN PROTEIN SYNTHESIS QUALITY CONTROL

ABSTRACT: Quality control defects of mitochondrial nascent chain synthesis trigger a sequential stress response characterised by OMA1 activation and ribosome decay, determining mitochondrial form and function.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Mus Musculus (mouse)

SUBMITTER: Tuula Nyman

LAB HEAD: Tuula Nyman

PROVIDER: PXD012416 | Pride | 2019-01-23

REPOSITORIES: Pride

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Publications

Mitochondrial stress response triggered by defects in protein synthesis quality control.

Richter Uwe U Ng Kah Ying KY Suomi Fumi F Marttinen Paula P Turunen Taina T Jackson Christopher C Suomalainen Anu A Vihinen Helena H Jokitalo Eija E Nyman Tuula A TA Isokallio Marita A MA Stewart James B JB Mancini Cecilia C Brusco Alfredo A Seneca Sara S Lombès Anne A Taylor Robert W RW Battersby Brendan J BJ

Life science alliance 20190125 1

Mitochondria have a compartmentalized gene expression system dedicated to the synthesis of membrane proteins essential for oxidative phosphorylation. Responsive quality control mechanisms are needed to ensure that aberrant protein synthesis does not disrupt mitochondrial function. Pathogenic mutations that impede the function of the mitochondrial matrix quality control protease complex composed of AFG3L2 and paraplegin cause a multifaceted clinical syndrome. At the cell and molecular level, defe ...[more]

PMID: 30683687

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