Mammalian Genome

, Volume 22, Issue 3, pp 139–147

How does the genetic assassin select its neuronal target?

  • James C. Stevens
  • Elizabeth M. C. Fisher
  • Simon Mead
Article

DOI: 10.1007/s00335-011-9319-5

Cite this article as:
Stevens, J.C., Fisher, E.M.C. & Mead, S. Mamm Genome (2011) 22: 139. doi:10.1007/s00335-011-9319-5

Abstract

Through many different routes of analysis, including human familial studies and animal models, we are identifying an increasing number of genes that are causative for human neurodegenerative disease and are now in a position for many such disorders to dissect the molecular pathology that gives rise to neuronal death. Yet a paradox remains: The majority of the genes identified cause neurodegeneration in specific neuronal subtypes, but the genes themselves are ubiquitously expressed. Furthermore, the different mutations in the same gene may cause quite different types of neurodegeneration. Something in our understanding of neurodegenerative disease is clearly missing, and we refer to this as the phenomenon of “neuronal targeting.” Here we discuss possible explanations for neuronal targeting, why specific neuronal subtypes are vulnerable to specific mutations in ubiquitously expressed genes.

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • James C. Stevens
    • 1
  • Elizabeth M. C. Fisher
    • 1
  • Simon Mead
    • 1
    • 2
  1. 1.Department of Neurodegenerative DiseaseUniversity College London, Queen SquareLondonUK
  2. 2.MRC Prion Unit, Institute of NeurologyUniversity College London, Queen SquareLondonUK

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