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oai:repisalud.isciii.es:20.500.12105/73492024-09-27T09:11:24Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605col_20.500.12105_19607

00925njm 22002777a 4500 dc Quirós, Pedro M author Español, Yaiza author Acin-Perez, Rebeca author Rodríguez, Francisco author Bárcena, Clea author Watanabe, Kenta author Calvo, Enrique author Loureiro, Marta author Fernández-García, M Soledad author Fueyo, Antonio author Vazquez, Jesus author Enriquez, Jose Antonio author López-Otín, Carlos author 2014-07-24 We generated mice deficient in Lon protease (LONP1), a major enzyme of the mitochondrial quality control machinery. Homozygous deletion of Lonp1 causes early embryonic lethality, whereas its haploinsufficiency protects against colorectal and skin tumors. Furthermore, LONP1 knockdown inhibits cellular proliferation and tumor and metastasis formation, whereas its overexpression increases tumorigenesis. Clinical studies indicate that high levels of LONP1 are a poor prognosis marker in human colorectal cancer and melanoma. Additionally, functional analyses show that LONP1 plays a key role in metabolic reprogramming by remodeling OXPHOS complexes and protecting against senescence. Our findings demonstrate the relevance of LONP1 for cellular and organismal viability and identify this protease as a central regulator of mitochondrial activity in oncogenesis. Cell Rep. 2014; 8(2):542-56 10.1016/j.celrep.2014.06.018 2211-1247 22111247 Cell reports 25017063 http://hdl.handle.net/20.500.12105/7349 ATP-dependent Lon protease controls tumor bioenergetics by reprogramming mitochondrial activity