Emerging role of Lon protease as a master regulator of mitochondrial functions

https://doi.org/10.1016/j.bbabio.2016.03.025Get rights and content
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Highlights

  • Lon protease is crucial for the maintenance of mitochondrial homeostasis.

  • Lon displays proteolytic activity, chaperone activity and mtDNA-binding activity.

  • Lon dysregulation is involved in cancer and in CODAS syndrome.

Abstract

Lon protease is a nuclear-encoded, mitochondrial ATP-dependent protease highly conserved throughout the evolution, crucial for the maintenance of mitochondrial homeostasis. Lon acts as a chaperone of misfolded proteins, and is necessary for maintaining mitochondrial DNA. The impairment of these functions has a deep impact on mitochondrial functionality and morphology. An altered expression of Lon leads to a profound reprogramming of cell metabolism, with a switch from respiration to glycolysis, which is often observed in cancer cells. Mutations of Lon, which likely impair its chaperone properties, are at the basis of a genetic inherited disease named of the cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome. This article is part of a Special Issue entitled ‘EBEC 2016: 19th European Bioenergetics Conference, Riva del Garda, Italy, July 2–6, 2016’, edited by Prof. Paolo Bernardi.

Keywords

Lon
Protease
Chaperone
Mitochondria
mtDNA
Cancer
CODAS syndrome

Cited by (0)

This article is part of a Special Issue entitled ‘EBEC 2016: 19th European Bioenergetics Conference, Riva del Garda, Italy, July 2–6, 2016’, edited by Prof. Paolo Bernardi.

1

Equally contributed to the paper.