LONP1

LONP1
已知的结构
PDB	直系同源搜索: PDBe RCSB
PDBID列表
	2X36
识别号
别名	LONP1；, LON, LONP, LonHS, PIM1, PRSS15, hLON, CODASS, lon peptidase 1, mitochondrial, Lon protease homolog, mitochondrial
外部ID	OMIM：605490 MGI：1921392 HomoloGene：3521 GeneCards：LONP1
相关疾病
	CODAS syndrome
基因位置（人类）
染色体	19号染色体
终止	5,720,572 bp
基因位置（小鼠）
染色体	小鼠17号染色体
终止	56,933,887 bp
RNA表达模式
	查阅更多表达数据
基因本体
分子功能	• 核苷酸结合; • 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结合; • ATP-dependent peptidase activity; • sequence-specific DNA binding; • single-stranded DNA binding;
细胞组分	• 膜; • 核质; • 线粒体基质; • 线粒体拟核; • 细胞核; • 线粒体; • 细胞质基质;
生物学过程	• 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;
	Sources:Amigo / QuickGO
直系同源
	9361
	74142
	ENSG00000196365
	ENSMUSG00000041168
	P36776
	Q8CGK3
	NM_001276479; NM_001276480; NM_004793
	NM_028782
	NP_001263408; NP_001263409; NP_004784
	NP_083058
	维基数据
查看/编辑人类	查看/编辑小鼠

线粒体Lon蛋白酶同源物（英语：Lon protease homolog, mitochondrial）是一个由人类LONP1基因编码的酶^[6]^[7]^[8]。

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^[8]。

参考文献

^ 與LONP1相關的疾病；在維基數據上查看/編輯參考.
^ ^2.0 ^2.1 ^2.2 GRCh38: Ensembl release 89: ENSG00000196365 - Ensembl, May 2017
^ ^3.0 ^3.1 ^3.2 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.
^ ^8.0 ^8.1 Entrez Gene: LONP1 lon peptidase 1, mitochondrial. （原始内容存档于2019-10-18）.

延伸阅读

{{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.

[1] 與LONP1相關的疾病；在維基數據上查看/編輯參考.

[refGRCh38Ensembl-2] 2.0 ^2.1 ^2.2 GRCh38: Ensembl release 89: ENSG00000196365 - Ensembl, May 2017

[refGRCm38Ensembl-3] 3.0 ^3.1 ^3.2 GRCm38: Ensembl release 89: ENSMUSG00000041168 - Ensembl, May 2017

[4] Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.

[5] Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid8248235-6] 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.

[pmid8119403-7] 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-8] 8.0 ^8.1 Entrez Gene: LONP1 lon peptidase 1, mitochondrial. （原始内容存档于2019-10-18）.

[6]

[7]

[8]

[1]

[2]

[3]

[4]

[5]