The neuronal ceroid lipofuscinoses: opportunities from model systems

Faller, K. M.E., Gutierrez Quintana, R., Mohammed, A., Rahim, A. A., Tuxworth, R. I., Wager, K. and Bond, M. (2015) The neuronal ceroid lipofuscinoses: opportunities from model systems. Biochimica et Biophysica Acta, 1852(10), pp. 2267-2278. (doi:10.1016/j.bbadis.2015.04.022) (PMID:25937302)

Full text not currently available from Enlighten.

Abstract

The neuronal ceroid lipofuscinoses are a group of severe and progressive neurodegenerative disorders, generally with childhood onset. Despite the fact that these diseases remain fatal, significant breakthroughs have been made in our understanding of the genetics that underpin these conditions. This understanding has allowed the development of a broad range of models to study disease processes, and to develop new therapeutic approaches. Such models have contributed significantly to our knowledge of these conditions. In this review we will focus on the advantages of each individual model, describe some of the contributions the models have made to our understanding of the broader disease biology and highlight new techniques and approaches relevant to the study and potential treatment of the neuronal ceroid lipofuscinoses. This article is part of a Special Issue entitled: "Current Research on the Neuronal Ceroid Lipofuscinoses (Batten Disease)".

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Faller, Ms Kiterie and Gutierrez Quintana, Mr Rodrigo
Authors: Faller, K. M.E., Gutierrez Quintana, R., Mohammed, A., Rahim, A. A., Tuxworth, R. I., Wager, K., and Bond, M.
College/School:College of Medical Veterinary and Life Sciences > School of Veterinary Medicine
Journal Name:Biochimica et Biophysica Acta
Publisher:Elsevier
ISSN:1388-1981
ISSN (Online):0006-3002
Published Online:29 April 2015

University Staff: Request a correction | Enlighten Editors: Update this record