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Faller KM, Gutierrez-Quintana R, Mohammed A, et al. The neuronal ceroid lipofuscinoses: Opportunities from model systems. Biochim Biophys Acta. 2015;1852(10):2267-78doi: 10.1016/j.bbadis.2015.04.022.
Faller, K. M., Gutierrez-Quintana, R., Mohammed, A., Rahim, A. A., Tuxworth, R. I., Wager, K., & Bond, M. (2015). The neuronal ceroid lipofuscinoses: Opportunities from model systems. Biochimica et biophysica acta, 1852(10), 2267-78. https://doi.org/10.1016/j.bbadis.2015.04.022
Faller, Kiterie M E, et al. "The neuronal ceroid lipofuscinoses: Opportunities from model systems." Biochimica et biophysica acta vol. 1852,10 (2015): 2267-78. doi: https://doi.org/10.1016/j.bbadis.2015.04.022
Faller KM, Gutierrez-Quintana R, Mohammed A, Rahim AA, Tuxworth RI, Wager K, Bond M. The neuronal ceroid lipofuscinoses: Opportunities from model systems. Biochim Biophys Acta. 2015 Oct;1852(10):2267-78. doi: 10.1016/j.bbadis.2015.04.022. Epub 2015 Apr 29. PMID: 25937302.
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Biochim Biophys Acta. 2015 Oct;1852(10):2267-78. doi: 10.1016/j.bbadis.2015.04.022. Epub 2015 Apr 29.

The neuronal ceroid lipofuscinoses: Opportunities from model systems.

Biochimica et biophysica acta

Kiterie M E Faller, Rodrigo Gutierrez-Quintana, Alamin Mohammed, Ahad A Rahim, Richard I Tuxworth, Kim Wager, Michael Bond

Affiliations

  1. School of Veterinary Medicine, College of Veterinary, Medical and Life Sciences, Bearsden Road, Glasgow G61 1QH, UK.
  2. College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UK.
  3. UCL School of Pharmacy, 29-39 Brunswick Square, London WC1N 1AX, UK.
  4. Cardiff School of Biosciences, Cardiff University, The Sir Martin Evans Building, Museum Avenue, Cardiff CF10 3AX, UK.
  5. MRC Laboratory for Molecular Cell Biology, University College of London, Gower Street, London WC1E 6BT, UK. Electronic address: [email protected].

PMID: 25937302 DOI: 10.1016/j.bbadis.2015.04.022

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)".

Copyright © 2015 Elsevier B.V. All rights reserved.

Keywords: Model systems; NCL; Neurodegeneration; Neuronal ceroid lipofuscinosis

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