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.
Emerging role of Lon protease as a master regulator of mitochondrial functions / Pinti, Marcello; Gibellini, Lara; Nasi, Milena; De Biasi, Sara; Bortolotti, Carlo Augusto; Iannone, Anna; Cossarizza, Andrea. - In: BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS. - ISSN 0005-2728. - STAMPA. - 1857:8(2016), pp. 1300-1306.
Data di pubblicazione: | 2016 |
Data di prima pubblicazione: | 28-mar-2016 |
Titolo: | Emerging role of Lon protease as a master regulator of mitochondrial functions |
Autore/i: | Pinti, Marcello; Gibellini, Lara; Nasi, Milena; De Biasi, Sara; Bortolotti, Carlo Augusto; Iannone, Anna; Cossarizza, Andrea |
Autore/i UNIMORE: | |
Digital Object Identifier (DOI): | http://dx.doi.org/10.1016/j.bbabio.2016.03.025 |
Rivista: | |
Volume: | 1857 |
Fascicolo: | 8 |
Pagina iniziale: | 1300 |
Pagina finale: | 1306 |
Codice identificativo ISI: | WOS:000378179300030 |
Codice identificativo Scopus: | 2-s2.0-84963516824 |
Codice identificativo Pubmed: | 27033304 |
Citazione: | Emerging role of Lon protease as a master regulator of mitochondrial functions / Pinti, Marcello; Gibellini, Lara; Nasi, Milena; De Biasi, Sara; Bortolotti, Carlo Augusto; Iannone, Anna; Cossarizza, Andrea. - In: BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS. - ISSN 0005-2728. - STAMPA. - 1857:8(2016), pp. 1300-1306. |
Tipologia | Articolo su rivista |
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2016 BBA Pinti.pdf | uncorrected proof | Post-print dell'autore (bozza post referaggio) | Administrator Richiedi una copia |
BBABIO_47641_edit_report.pdf | Post-print dell'autore (bozza post referaggio) | Open Access Visualizza/Apri |

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