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      The nuclear proteasome and the degradation of oxidatively damaged proteins.

      Amino Acids
      Animals, Cell Nucleus, enzymology, Humans, Multienzyme Complexes, metabolism, Neurodegenerative Diseases, etiology, Nuclear Proteins, Oxidation-Reduction, Oxidative Stress, physiology, Poly(ADP-ribose) Polymerases, Proteasome Endopeptidase Complex

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          Abstract

          The accumulation of oxidized proteins is known to be linked to some severe neurodegenerative diseases like Alzheimer's, Parkinson's and Huntington's disease. Furthermore, the aging process is also accompanied by an ongoing aggregation of misfolded and damaged proteins. Therefore, mammalian cells have developed potent degradation systems, which selectively degrade damaged and misfolded proteins. The proteasomal system is largely responsible for the removal of oxidatively damaged proteins form the cellular environment. Not only cytosolic proteins are prone to oxidative stress, also nuclear proteins are readily oxidized. The nuclear proteasomal system is responsible for the degradation of these proteins. This review is focused on the specific degradation of oxidized nuclear proteins, the role of the proteasome in this process and the regulation of the nuclear proteasomal system under oxidative conditions.

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