Journal article

LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins

Ayenachew Bezawork-Geleta, Erica J Brodie, David A Dougan, Kaye N Truscott

Scientific Reports | Springer Science and Business Media LLC | Published : 2015

Abstract

Maintenance of mitochondrial protein homeostasis is critical for proper cellular function. Under normal conditions resident molecular chaperones and proteases maintain protein homeostasis within the organelle. Under conditions of stress however, misfolded proteins accumulate leading to the activation of the mitochondrial unfolded protein response (UPRmt). While molecular chaperone assisted refolding of proteins in mammalian mitochondria has been well documented, the contribution of AAA+ proteases to the maintenance of protein homeostasis in this organelle remains unclear. To address this gap in knowledge we examined the contribution of human mitochondrial matrix proteases, LONM and CLPXP, to..

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Grants

Awarded by Australian Research Council Fellowship


Funding Acknowledgements

This work was supported by Australian Research Council Fellowships to K.N.T. (FT0992033) and D.A.D (DP110103936), a La Trobe University Postgraduate Research Scholarship to A.B-G and an Australian Postgraduate Award to E.J.B. We thank Lauri Fisher for technical assistance with the immunoprecipitation experiment, N.J. Hoogenraad (La Trobe University, Melbourne) for OTC plasmid and antiserum and M.T. Ryan (Monash University, Melbourne) for NDUFA9 antiserum.