LONP1

Lon peptidase 1, mitochondrial
Identifiers
Symbols	LONP1 ; PIM1; LON; LONP; LonHS; MGC1498; PRSS15; hLON
External IDs	Template:OMIM5 Template:MGI HomoloGene: 3521
Gene ontology Molecular function Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Cellular component Template:GNF GO Biological process Template:GNF GO Template:GNF GO
RNA expression pattern
File:PBB GE LONP1 209017 s at tn.png
More reference expression data
Orthologs
Template:GNF Ortholog box
Species	Human	Mouse
Entrez	n/a	n/a
Ensembl	n/a	n/a
UniProt	n/a	n/a
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	n/a
Location (UCSC)	n/a	n/a
PubMed search	n/a	n/a

Lon peptidase 1, mitochondrial, also known as LONP1, is a human gene.

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.

Further reading

• Lu B, Yadav S, Shah PG; et al. (2007). "Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance". J. Biol. Chem. 282 (24): 17363–74. doi:10.1074/jbc.M611540200. PMID 17420247.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Bota DA, Ngo JK, Davies KJ (2005). "Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death". Free Radic. Biol. Med. 38 (5): 665–77. doi:10.1016/j.freeradbiomed.2004.11.017. PMID 15683722.CS1 maint: Multiple names: authors list (link)
• Gerhard DS, Wagner L, Feingold EA; et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMID 15489334.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• 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. PMID 14739292.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• 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. PMID 14702039.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• 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". Gene. 306: 45–55. PMID 12657466.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Strausberg RL, Feingold EA, Grouse LH; et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMID 12477932.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Bota DA, Davies KJ (2002). "Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism". Nat. Cell Biol. 4 (9): 674–80. doi:10.1038/ncb836. PMID 12198491.
• Hori O, Ichinoda F, Tamatani T; et al. (2002). "Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease". J. Cell Biol. 157 (7): 1151–60. doi:10.1083/jcb.200108103. PMID 12082077.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Fu GK, Markovitz DM (1998). "The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner". Biochemistry. 37 (7): 1905–9. doi:10.1021/bi970928c. PMID 9485316.
• Korenberg JR, Chen XN, Adams MD, Venter JC (1996). "Toward a cDNA map of the human genome". Genomics. 29 (2): 364–70. PMID 8666383.CS1 maint: Multiple names: authors list (link)
• Wang N, Gottesman S, Willingham MC; et al. (1994). "A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease". Proc. Natl. Acad. Sci. U.S.A. 90 (23): 11247–51. PMID 8248235.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Amerik AYu , Petukhova GV, Grigorenko VG; et al. (1994). "Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases". FEBS Lett. 340 (1–2): 25–8. PMID 8119403.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Wang N, Maurizi MR, Emmert-Buck L, Gottesman MM (1994). "Synthesis, processing, and localization of human Lon protease". J. Biol. Chem. 269 (46): 29308–13. PMID 7961901.CS1 maint: Multiple names: authors list (link)