Journal of Neural Transmission

, Volume 118, Issue 1, pp 27–45 | Cite as

Transgenic animal models of neurodegeneration based on human genetic studies

  • Brandon K. Harvey
  • Christopher T. Richie
  • Barry J. Hoffer
  • Mikko Airavaara
Basic Neurosciences, Genetics and Immunology - Review Article

Abstract

The identification of genes linked to neurodegenerative diseases such as Alzheimer’s disease (AD), amyotrophic lateral sclerosis (ALS), Huntington’s disease (HD) and Parkinson’s disease (PD) has led to the development of animal models for studying mechanism and evaluating potential therapies. None of the transgenic models developed based on disease-associated genes have been able to fully recapitulate the behavioral and pathological features of the corresponding disease. However, there has been enormous progress made in identifying potential therapeutic targets and understanding some of the common mechanisms of neurodegeneration. In this review, we will discuss transgenic animal models for AD, ALS, HD and PD that are based on human genetic studies. All of the diseases discussed have active or complete clinical trials for experimental treatments that benefited from transgenic models of the disease.

Keywords

ALS PD AD HD Huntington’s disease Parkinson’s disease Amyotrophic lateral sclerosis Alzheimer’s disease Transgenic Animal model Neurodegeneration Genetic linkage 

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Copyright information

© Springer-Verlag (outside the USA) 2010

Authors and Affiliations

  • Brandon K. Harvey
    • 1
  • Christopher T. Richie
    • 1
  • Barry J. Hoffer
    • 1
  • Mikko Airavaara
    • 1
  1. 1.Intramural Research ProgramNational Institute on Drug Abuse, National Institutes of HealthBaltimoreUSA

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