LONP1

From Wikipedia, the free encyclopedia

Lon peptidase 1, mitochondrial

Identifiers

LONP1; PIM1; LON; LONP; LonHS; MGC1498; PRSS15; hLON

External IDs

OMIM: 605490 MGI: 1921392 HomoloGene: 3521

Gene ontology
Molecular Function:	• nucleotide binding • DNA binding • ATP-dependent peptidase activity • serine-type endopeptidase activity • ATP binding • nucleoside-triphosphatase activity
Cellular Component:	• mitochondrion
Biological Process:	• ATP-dependent proteolysis • defense response

RNA expression pattern

More reference expression data

Orthologs

Human

Mouse

Refseq

NM_004793 (mRNA)
NP_004784 (protein)

NM_028782 (mRNA)
NP_083058 (protein)

Location

Chr 19: 5.64 - 5.67 Mb

Chr 17: 56.3 - 56.31 Mb

Pubmed search

[1]

[2]

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

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.^[1]

[edit] References

^ ^a ^b Entrez Gene: LONP1 lon peptidase 1, mitochondrial.

[edit] 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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.