LONP1

LONP1
已知的结构 PDB 直系同源搜索：PDBe RCSB PDBID列表 2X36
标识符
代号	LONP1
扩展标识	OMIM：605490；MGI：1921392；HomoloGene：3521；GeneCards：LONP1；OMA：LONP1 - orthologs
EC编号	3.4.21.53
基因位置（人类） 染色体 19號染色體 基因座 19p13.3 起始 5,691,834 bp 终止 5,720,572 bp
基因位置（小鼠） 染色体 小鼠17号染色体 基因座 17|17 D 起始 56,921,297 bp 终止 56,933,887 bp
RNA表达模式 Bgee 人类 小鼠（直系同源） Top expressed in right adrenal gland right adrenal cortex left adrenal gland left adrenal cortex apex of heart stromal cell of endometrium right lobe of liver muscle of thigh Top expressed in Ileal epithelium right kidney muscle of thigh left lobe of liver 查阅更多表达数据 BioGPS 查阅更多表达数据
基因本體 分子功能 核苷酸結合 DNA结合 mitochondrial promoter sequence-specific DNA binding ADP binding DNA polymerase binding G-quadruplex DNA binding 肽酶活性 ATPase activity 血浆蛋白结合 single-stranded RNA binding serine-type peptidase activity serine-type endopeptidase activity 水解酶活性 ATP結合 ATP-dependent peptidase activity sequence-specific DNA binding single-stranded DNA binding 細胞組分 膜 核质 线粒体基质 线粒体拟核 细胞核 線粒體 细胞质基质 生物學過程 mitochondrial DNA metabolic process mitochondrial genome maintenance 老化 mitochondrion organization 蛋白酶解 protein catabolic process response to aluminum ion response to hormone proteolysis involved in cellular protein catabolic process protein homooligomerization protein quality control for misfolded or incompletely synthesized proteins response to hypoxia cellular response to oxidative stress oxidation-dependent protein catabolic process chaperone-mediated protein complex assembly 来源：Amigo / QuickGO
直系同源 物種 人類 小鼠 Entrez 9361 74142 Ensembl ENSG00000196365 ENSMUSG00000041168 UniProt P36776 Q8CGK3 mRNA序列 ​NM_001276479 ​NM_001276480 ​NM_004793 NM_028782 蛋白序列 NP_001263408 ​NP_001263409 ​NP_004784 NP_083058 基因位置 Chr 19：5.69 – 5.72 Mb Chr 17：56.92 – 56.93 Mb PubMed查询
维基数据
檢視/編輯人類 檢視/編輯小鼠

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。

延伸阅读

• 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.