basic FUNCTION
| regulator of mitochondrial DNA replication and for gene expression |
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participates directly in the metabolism of mtDNA |
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required for normal survival and proliferation |
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degrades folded proteins and initiates substrate cleavage non-processively |
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recognizes specific surface determinants or folds, initiates proteolysis at solvent-accessible sites, and generates unfolded polypeptides that are then processively degraded |
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potential roles for LONP1 in linking protein and mtDNA quality control |
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seems to play a major role in the elimination of oxidatively modified proteins in the mitochondrial matrix |
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ATP-powered protease that binds DNA |
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LONP1 and CLPP have been shown to degrade unfolded and damaged proteins in the matrix of mitochondria |
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regulates mitochondrial transcription by stabilizing the mitochondrial TFAM/mtDNA ratio via selective degradation of TFAM |
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modulates mtDNA biogenesis by the selective degradation of TFAM |
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nuclear-encoded mitochondrial enzyme, degrades oxidized proteins of the mitochondrial (mt) matrix, and participates in the replication of mtDNA |
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can be considered a stress responsive protein |
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ATP-dependent protease that controls the selective turnover of mitochondrial matrix proteins |
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nuclearly encoded and mitochondrially located stress-responsive protease, that is involved in heme-mediated ALAS1 turnover |
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has a wide variety of targets and is likely to play different roles depending of the cell type |
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plays a key role in metabolic reprogramming by remodeling OXPHOS complexes and protecting against senescence |
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independent of its proteolytic activity, LONP1 influences function of the mitochondrial genome and respiratory chain |
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importance of mitochondrial Lon (LONP1) in relation to oxidative stress and aging |
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may also contribute, albeit indirectly, to transcriptional regulation within the mitochondria |
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plays an important role in maintaining mitochondrial integrity during aging and aging-related diseases |
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LONP1, LONP2 are in high concentration and close proximity to proteins that are at risk of oxidative damage and have the need for rapid degradation |
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is a nuclear-encoded mitochondrial protease |