Sandbox Reserved 1644: Difference between revisions
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== Disease == | == Disease == | ||
<p align="justify">Various [https://en.wikipedia.org/wiki/Myopathy myopathy], [https://en.wikipedia.org/wiki/Type_2_diabetes type 2 diabetes], [https://en.wikipedia.org/wiki/Parkinson%27s_disease Parkinson's disease], or [https://en.wikipedia.org/wiki/Alzheimer%27s_disease Alzheimer's disease] are human [https://en.wikipedia.org/wiki/Degenerative_disease degenerative disease] partly due to abnormalities of the mitochondria<ref>Wang, N, S Gottesman, M C Willingham, M M Gottesman, and M R Maurizi. “A Human Mitochondrial ATP-Dependent Protease That Is Highly Homologous to Bacterial Lon Protease.” Proceedings of the National Academy of Sciences 90, no. 23 (December 1, 1993): 11247–51. https://doi.org/10.1073/pnas.90.23.11247. | |||
</ref>. In fact, Lon protease has a role in [https://en.wikipedia.org/wiki/Cancer cancer], [https://en.wikipedia.org/wiki/Apoptosis apoptosis] and [https://en.wikipedia.org/wiki/Ageing aging] because this protein is an essential part of developmental pathways and stress response. | |||
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[https://en.wikipedia.org/wiki/Mutant Mutant] in Lon decreases the degradation capacity of proteins with abnormal conformations which lead to mitochondrial dysfunction. Mitochondrial dysfunction causes normal cells to become apoptotic, or to aberrant adaptation and selection of hypoxic phenotypes in pathological conditions like cancer<ref>Bota, Daniela A., and Kelvin J. A. Davies. “Mitochondrial 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>. | |||
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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>. | |||
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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. | |||
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A [https://en.wikipedia.org/wiki/Consensus_sequence consensus] binding site of Nuclear Respiratory Factor 2 (NRF-2) is present on the region -623/+1 of the LONP1 promoter which is important for response to [https://en.wikipedia.org/wiki/Reactive_oxygen_species reactive oxygen species] related to oxidative stress. | |||
This is a sample scene created with SAT to <scene name="/12/3456/Sample/1">color</scene> by Group, and another to make <scene name="/12/3456/Sample/2">a transparent representation</scene> of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes. | This is a sample scene created with SAT to <scene name="/12/3456/Sample/1">color</scene> by Group, and another to make <scene name="/12/3456/Sample/2">a transparent representation</scene> of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes. | ||
