RESEARCH ARTICLE


Protein Aggregation in the Cell Nucleus: Structure, Function and Topology



Anna von Mikecz*
Institut für umweltmedizinische Forschung (IUF) at Heinrich-Heine-University Düsseldorf, Auf'm Hennekamp 50, 40225 Düsseldorf, Germany


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Creative Commons License
© 2009 Anna von Mikecz

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: (https://creativecommons.org/licenses/by/4.0/legalcode). This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* 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


Abstract

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.