Sandbox Reserved 1644: Difference between revisions

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</ref>. This protein has three isoforms obtained by [https://en.wikipedia.org/wiki/Alternative_splicing alternative splicing] of the portion of DNA coding for this protein <ref>He, Lihong, Dongyang Luo, Fan Yang, Chunhao Li, Xuegong Zhang, Haiteng Deng, et Jing-Ren Zhang. « Multiple domains of bacterial and human Lon proteases define substrate selectivity ». Emerging Microbes & Infections 7 (17 août 2018). https://doi.org/10.1038/s41426-018-0148-4.
</ref>. This protein has three isoforms obtained by [https://en.wikipedia.org/wiki/Alternative_splicing alternative splicing] of the portion of DNA coding for this protein <ref>He, Lihong, Dongyang Luo, Fan Yang, Chunhao Li, Xuegong Zhang, Haiteng Deng, et Jing-Ren Zhang. « Multiple domains of bacterial and human Lon proteases define substrate selectivity ». Emerging Microbes & Infections 7 (17 août 2018). https://doi.org/10.1038/s41426-018-0148-4.
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Globally there is a great diversity of Lon proteins, but they are all organised in an oligomeric ring structure, mostly hexameric structure with identical subunits.
Globally there is a great diversity of Lon proteins, but they are all organized in an oligomeric ring structure, mostly hexameric structure with identical subunits.
Lon proteins are therefore an hexameric chambered [https://en.wikipedia.org/wiki/Protease protease] complex. (This structure is similar with yeast [https://www.yeastgenome.org/locus/S000000118 Pim1] )
Lon proteins are therefore an hexameric chambered [https://en.wikipedia.org/wiki/Protease protease] complex. (This structure is similar to yeast [https://www.yeastgenome.org/locus/S000000118 Pim1] )
The six Lon monomers are forming three pairs of legs owned by the N-terminal domain of the protein. This structure is emerging of the protein as a trimer of dimers <ref>Kutejová, Eva. « Mitochondrial Lon protease-unique structure and essential function in mammalian cells ». Integrative Cancer Science and Therapeutics 5, nᵒ 6 (2018). https://doi.org/10.15761/ICST.1000296.
The six Lon monomers are forming three pairs of legs owned by the N-terminal domain of the protein. This structure is emerging of the protein as a trimer of dimers <ref>Kutejová, Eva. « Mitochondrial Lon protease-unique structure and essential function in mammalian cells ». Integrative Cancer Science and Therapeutics 5, nᵒ 6 (2018). https://doi.org/10.15761/ICST.1000296.
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Like many proteins, Lon is a '''flexible''' peptide which has different '''three-dimensional conformations'''.  The protein can therefore pass from one conformation to another by hydrolysis of [https://biologydictionary.net/atp/ ATP]<ref>Voos, Wolfgang, et Karen Pollecker. « The Mitochondrial Lon Protease: Novel Functions off the Beaten Track? » Biomolecules 10, nᵒ 2 (7 février 2020). https://doi.org/10.3390/biom10020253.
Like many proteins, Lon is a '''flexible''' peptide which has different '''three-dimensional conformations'''.  The protein can therefore pass from one conformation to another by hydrolysis of [https://biologydictionary.net/atp/ ATP]<ref>Voos, Wolfgang, et Karen Pollecker. « The Mitochondrial Lon Protease: Novel Functions off the Beaten Track? » Biomolecules 10, nᵒ 2 (7 février 2020). https://doi.org/10.3390/biom10020253.
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Lon expression is necessary for survival in mammals. Indeed, a [https://en.wikipedia.org/wiki/Zygosity#Homozygous homozygous] [https://en.wikipedia.org/wiki/Deletion_(genetics) deletion] of LONP1 is lethal for early embryonic<ref>Bota, Daniela A., and Kelvin J. A. Davies. “Mhttps://proteopedia.org/wiki/skins/common/images/button_extlink.pngitochondrial Lon Protease in Human Disease and Aging: Including an Etiologic Classification of Lon-Related Diseases and Disorders.” Free Radical Biology & Medicine 100 (November 2016): 188–98. https://doi.org/10.1016/j.freeradbiomed.2016.06.031.</ref>. Indeed, the [https://rarediseases.info.nih.gov/diseases/1418/codas-syndrome#:~:text=Summary,-Listen&text=Codas%20syndrome%20is%20a%20multiple,Dental%2C%20Auricular%20and%20Skeletal%20anomalies.&text=To%20date%2C%20three%20affected%20children,from%20Brazil)%20have%20been%20reported. CODAS Syndrome] is a rare and multi-system developmental disorder from heterozygous or homozygous mutations in LONP1 where all the affected children were very severely impacted by their disease.
Lon expression is necessary for survival in mammals. Indeed, a [https://en.wikipedia.org/wiki/Zygosity#Homozygous homozygous] [https://en.wikipedia.org/wiki/Deletion_(genetics) deletion] of LONP1 is lethal for early embryonic<ref>Bota, Daniela A., and Kelvin J. A. Davies. “Mhttps://proteopedia.org/wiki/skins/common/images/button_extlink.pngitochondrial Lon Protease in Human Disease and Aging: Including an Etiologic Classification of Lon-Related Diseases and Disorders.” Free Radical Biology & Medicine 100 (November 2016): 188–98. https://doi.org/10.1016/j.freeradbiomed.2016.06.031.</ref>. Indeed, the [https://rarediseases.info.nih.gov/diseases/1418/codas-syndrome#:~:text=Summary,-Listen&text=Codas%20syndrome%20is%20a%20multiple,Dental%2C%20Auricular%20and%20Skeletal%20anomalies.&text=To%20date%2C%20three%20affected%20children,from%20Brazil)%20have%20been%20reported. CODAS Syndrome] is a rare and multi-system developmental disorder from heterozygous or homozygous mutations in LONP1 where all the affected children were very severely impacted by their disease.
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The LONP1 gene is regulated, when the cell undergoes a '''heat shock''', [https://en.wikipedia.org/wiki/Starvation starvation] or oxidative stress the gene is '''up-regulated'''. On the contrary, Lon is '''down-regulated''' with aging, extensive [https://en.wikipedia.org/wiki/Hypoxia hypoxia], and prolonged oxidative stress. So Lon is an important factor in aging and degenerative disease.  
The LONP1 gene is regulated, when the cell undergoes a '''heat shock''', [https://en.wikipedia.org/wiki/Starvation starvation] or oxidative stress the gene is '''up-regulated'''. On the contrary, Lon is '''down-regulated''' with aging, extensive [https://en.wikipedia.org/wiki/Hypoxia hypoxia], and prolonged oxidative stress. So Lon is an important factor in aging and degenerative disease.  
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