粒線體Lon蛋白酶同源物(英語:Lon protease homolog, mitochondrial)是一個由人類LONP1基因編碼的酶[6][7][8]。
Quick Facts LONP1, 已知的結構 ...
LONP1 |
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識別號 |
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別名 | LONP1;, LON, LONP, LonHS, PIM1, PRSS15, hLON, CODASS, lon peptidase 1, mitochondrial, Lon protease homolog, mitochondrial |
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外部ID | OMIM:605490 MGI:1921392 HomoloGene:3521 GeneCards:LONP1 |
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相關疾病 |
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CODAS syndrome[1] |
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RNA表現模式 |
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![](//upload.wikimedia.org/wikipedia/commons/thumb/b/b5/PBB_GE_LONP1_209017_s_at_fs.png/640px-PBB_GE_LONP1_209017_s_at_fs.png) |
查閱更多表現資料 |
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直系同源 |
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物種 | 人類 | 小鼠 |
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Entrez | | |
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Ensembl | | |
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UniProt | | |
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mRNA序列 | | |
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蛋白序列 | | |
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基因位置(UCSC) | Chr 19: 5.69 – 5.72 Mb | Chr 17: 56.92 – 56.93 Mb |
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PubMed查找 | [4] | [5] |
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維基資料 |
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這個基因編碼的粒線體基質蛋白質屬於ATP依賴的Lon蛋白酶家族,類似於大腸桿菌基因編碼的特異性調控目標蛋白降解的蛋白。這種蛋白質結合在粒線體基因組啟動子的輕鏈和重鏈的特異性序列上,參與DNA複製和轉錄的調控[8]。
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.
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- 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.