Lon protease homolog, mitochondrial is an enzyme that in humans is encoded by the LONP1gene.
This gene encodes a mitochondrial matrix protein in the Lon family of ATP-dependent proteases. A similar E. coli protein regulates gene expression by targeting specific regulatory proteins for degradation. This protein binds a specific sequence in the light and heavy chain promoters of the mitochondrial genome which are involved in regulation of DNA replication and transcription.
Liu T, Lu B, Lee I et al. (2004). "DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate". J. Biol. Chem.279 (14): 13902–10. doi:10.1074/jbc.M309642200. PMID14739292.
Ota T, Suzuki Y, Nishikawa T et al. (2004). "Complete sequencing and characterization of 21,243 full-length human cDNAs". Nat. Genet.36 (1): 40–5. doi:10.1038/ng1285. PMID14702039.
Lu B, Liu T, Crosby JA et al. (2003). "The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals". Gene306: 45–55. doi:10.1016/S0378-1119(03)00403-7. PMID12657466.
Fu GK, Markovitz DM (1998). "The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner". Biochemistry37 (7): 1905–9. doi:10.1021/bi970928c. PMID9485316.