TOBIOJ The Open Biology Journal Open Bio. J. 1874-1967 Bentham Open TOBIOJ-2-193 10.2174/1874196700902010193 Biology Protein Aggregation in the Cell Nucleus: Structure, Function and Topology von Mikecz Anna * Institut für umweltmedizinische Forschung (IUF) at Heinrich-Heine-University Düsseldorf, Auf'm Hennekamp 50, 40225 Düsseldorf, Germany Address correspondence to this author at the Institut fuer umweltmedizinische Forschung (IUF) at Heinrich-Heine-University Düsseldorf, Auf'm Hennekamp 50, 40225 Düsseldorf, Germany; Tel: ++49-211-3389-358; E-mail: mikecz@tec-source.de; Web: www.tec-source.de/mikecz 31 12 2009 2009 2 193 199 10 04 2009 04 06 2009 05 06 2009 © 2009 Anna von Mikecz 2009 Anna von Mikecz

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The nucleus represents a cellular control unit that regulates all events concerning the storage and processing of DNA and RNA. It is organized by highly crowded, dynamic assemblies of proteins and nucleic acids in molecular machines, ribonucleoprotein complexes, clusters of ongoing nuclear processes, nuclear bodies, and chromatin. This review discusses the occurrence of nuclear protein aggregation with special emphasis on the functional architecture of the nucleus, and quality control by the ubiquitin-proteasome system.

 Keywords Amyloid gene expression Huntington's disease neurodegeneration nucleus polyglutamine protein aggregation trinucleotide repeat ubiquitin-proteasome system