Mitochondrial Lon protease-unique structure and essential function in mammalian cells

Lon protease belongs to the family of ATP dependent proteases. The Lon protease was first characterized by Prof. Alfred Goldberg in E. coli in 1981 [1]. Since then, it has been found in almost all living cells except some parasites like Trypanosoma brucei. In eukaryotes Lon is localized mainly in mitochondria, however peroxisomes as well as chloroplasts have their own Lon proteases. Although several eukaryotes can exist without Lon protease, for mammals it is an essential enzyme whose dysfunction has been identified in a number of diseases. Downregulation of this key mitochondrial component was observed in association with aging. Lon protease plays an important role also in malignant transformation that requires the adaptation to new sources of energy and is connected to hypoxia. This is the reason why Lon is highly expressed in aggressive tumors [2].