The Open Biology Journal

2009, 2 : 193-199
Published online 2009 December 31. DOI: 10.2174/1874196700902010193
Publisher ID: TOBIOJ-2-193

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

Anna von Mikecz, *

* 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: ; Web: mikecz@tec-source.de

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.