Table of Contents
New Journal of Science
Volume 2014 (2014), Article ID 468306, 18 pages
http://dx.doi.org/10.1155/2014/468306
Review Article

Nucleoporin Gene Fusions and Hematopoietic Malignancies

Institute for Molecular Biology and Medicine, Université Libre de Bruxelles, rue Profs. Jeener et Brachet 12, 6041 Charleroi, Belgium

Received 7 April 2014; Accepted 9 May 2014; Published 27 May 2014

Academic Editor: Brij B. Singh

Copyright © 2014 Birthe Fahrenkrog. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Nuclear pore complexes (NPCs) are the sole gateways between the nucleus and the cytoplasm of eukaryotic cells and they mediate all macromolecular trafficking between these cellular compartments. Nucleocytoplasmic transport is highly selective and precisely regulated and as such an important aspect of normal cellular function. Defects in this process or in its machinery have been linked to various human diseases, including cancer. Nucleoporins, which are about 30 proteins that built up NPCs, are critical players in nucleocytoplasmic transport and have also been shown to be key players in numerous other cellular processes, such as cell cycle control and gene expression regulation. This review will focus on the three nucleoporins Nup98, Nup214, and Nup358. Common to them is their significance in nucleocytoplasmic transport, their multiple other functions, and being targets for chromosomal translocations that lead to haematopoietic malignancies, in particular acute myeloid leukaemia. The underlying molecular mechanisms of nucleoporin-associated leukaemias are only poorly understood but share some characteristics and are distinguished by their poor prognosis and therapy outcome.