LONP1

线粒体Lon蛋白酶同源物（英語：Lon protease homolog, mitochondrial）是一个由人类LONP1基因编码的酶。 这个基因编码的线粒体基质蛋白质属于ATP依赖的Lon蛋白酶家族，类似于大肠杆菌基因编码的特异性调控目标蛋白降解的蛋白。这种蛋白质结合在线粒体基因组启动子的轻链和重链的特异性序列上，参与DNA复制和转录的调控。 與LONP1相關的疾病；在維基數據上查看/編輯參考.  GRCh38: Ensembl release 89: ENSG00000196365 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000041168 - Ensembl, May 2017 Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.  Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.  Wang N, Gottesman S, Willingham MC, Gottesman MM, Maurizi MR. A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease. Proc Natl Acad Sci U S A. Jan 1994, 90 (23): 11247–51. PMC 47959 . PMID 8248235. doi:10.1073/pnas.90.23.11247.  Amerik AYu, Petukhova GV, Grigorenko VG, Lykov IP, Yarovoi SV, Lipkin VM, Gorbalenya AE. Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases. FEBS Lett. Apr 1994, 340 (1–2): 25–8. PMID 8119403. doi:10.1016/0014-5793(94)80166-5.  Entrez Gene: LONP1 lon peptidase 1, mitochondrial. （原始内容存档于2019-10-18）.  {{refbegin} Lu B; Yadav S; Shah PG; et al. Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance. J. Biol. Chem. 2007, 282 (24): 17363–74. PMID 17420247. doi:10.1074/jbc.M611540200.  Bota DA, Ngo JK, Davies KJ. Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death. Free Radic. Biol. Med. 2005, 38 (5): 665–77. PMID 15683722. doi:10.1016/j.freeradbiomed.2004.11.017.  Gerhard DS; Wagner L; Feingold EA; et al. The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC). Genome Res. 2004, 14 (10B): 2121–7. PMC 528928 . PMID 15489334. doi:10.1101/gr.2596504.  Liu T; Lu B; Lee I; et al. DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate. J. Biol. Chem. 2004, 279 (14): 13902–10. PMID 14739292. doi:10.1074/jbc.M309642200.  Ota T; Suzuki Y; Nishikawa T; et al. Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. 2004, 36 (1): 40–5. PMID 14702039. doi:10.1038/ng1285.  Lu B; Liu T; Crosby JA; et al. The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals. Gene. 2003, 306: 45–55. PMID 12657466. doi:10.1016/S0378-1119(03)00403-7.  Strausberg RL; Feingold EA; Grouse LH; et al. Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proc. Natl. Acad. Sci. U.S.A. 2003, 99 (26): 16899–903. PMC 139241 . PMID 12477932. doi:10.1073/pnas.242603899.  Bota DA, Davies KJ. Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism. Nat. Cell Biol. 2002, 4 (9): 674–80. PMID 12198491. doi:10.1038/ncb836.  Hori O; Ichinoda F; Tamatani T; et al. Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease. J. Cell Biol. 2002, 157 (7): 1151–60. PMC 2173558 . PMID 12082077. doi:10.1083/jcb.200108103.  Fu GK, Markovitz DM. The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner. Biochemistry. 1998, 37 (7): 1905–9. PMID 9485316. doi:10.1021/bi970928c.  Korenberg JR, Chen XN, Adams MD, Venter JC. Toward a cDNA map of the human genome. Genomics. 1996, 29 (2): 364–70. PMID 8666383. doi:10.1006/geno.1995.9993.  Wang N, Maurizi MR, Emmert-Buck L, Gottesman MM. Synthesis, processing, and localization of human Lon protease. J. Biol. Chem. 1994, 269 (46): 29308–13. PMID 7961901.