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Lon peptidase 1, mitochondrial
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols LONP1 ; LON; LONP; LonHS; PIM1; PRSS15; hLON
External IDs OMIM605490 MGI1921392 HomoloGene3521 GeneCards: LONP1 Gene
RNA expression pattern
PBB GE LONP1 209017 s at tn.png
More reference expression data
Species Human Mouse
Entrez 9361 74142
Ensembl ENSG00000196365 ENSMUSG00000041168
UniProt P36776 Q8CGK3
RefSeq (mRNA) NM_001276479 NM_028782
RefSeq (protein) NP_001263408 NP_083058
Location (UCSC) Chr 19:
5.69 – 5.72 Mb
Chr 17:
56.61 – 56.63 Mb
PubMed search [1] [2]

Lon protease homolog, mitochondrial is an enzyme that in humans is encoded by the LONP1 gene.[1][2][3]

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.[3]


  1. ^ Wang N, Gottesman S, Willingham MC, Gottesman MM, Maurizi MR (Jan 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. doi:10.1073/pnas.90.23.11247. PMC 47959. PMID 8248235. 
  2. ^ Amerik AYu, Petukhova GV, Grigorenko VG, Lykov IP, Yarovoi SV, Lipkin VM, Gorbalenya AE (Apr 1994). "Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases". FEBS Lett 340 (1–2): 25–8. doi:10.1016/0014-5793(94)80166-5. PMID 8119403. 
  3. ^ a b "Entrez Gene: LONP1 lon peptidase 1, mitochondrial". 

Further reading[edit]