Table of Contents
ISRN Molecular Biology
Volume 2014, Article ID 190976, 5 pages
http://dx.doi.org/10.1155/2014/190976
Review Article

Recent Trends in Detection of Huntingtin and Preclinical Models of Huntington’s Disease

Department of Pharmaceutical Sciences, Butler University, 4600 Sunset Avenue, Indianapolis, IN 46208, USA

Received 11 February 2014; Accepted 20 March 2014; Published 14 May 2014

Academic Editors: M. Harata and T. Straub

Copyright © 2014 Neelima Mantha et al. 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

Huntington’s disease is a genetically inherited neurodegenerative disease that is characterized by neuronal cell death in the brain. Molecular biology techniques to detect and quantify huntingtin protein in biological samples involve fluorescence imaging, western blotting, and PCR. Modified cell lines are widely used as models for Huntington’s disease for preclinical screening of drugs to study their ability to suppress the expression of huntingtin. Although worm and fly species have been experimented on as models for Huntington’s disease, the most successful animal models have been reported to be primates. This review critically analyses the molecular biology techniques for detection and quantitation of huntingtin and evaluates the various animal species for use as models for Huntington’s disease.