2. Academic Validation
  3. Emerging role of Lon protease as a master regulator of mitochondrial functions

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

  • Biochim Biophys Acta. 2016 Aug;1857(8):1300-1306. doi: 10.1016/j.bbabio.2016.03.025.
Marcello Pinti 1,  Lara Gibellini 2,  Milena Nasi 2,  Sara De Biasi 2,  Carlo Augusto Bortolotti 1,  Anna Iannone 3,  Andrea Cossarizza 4
Affiliations

Affiliations

  • 1 Department of Life Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  • 2 Department of Surgery, Medicine, Dentistry and Morphological Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  • 3 Department of Diagnostics, Clinical and Public Health Medicine, University of Modena and Reggio Emilia, Modena, Italy.
  • 4 Department of Surgery, Medicine, Dentistry and Morphological Sciences, University of Modena and Reggio Emilia, Modena, Italy. Electronic address: [email protected].
PMID: 27033304 DOI: 10.1016/j.bbabio.2016.03.025
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

CODAS syndrome; Cancer; Chaperone; Lon; Mitochondria; Protease; mtDNA.

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