The genetics of neurodegenerative diseases

Authors: Hardy, John; Orr, Harry

Source: Journal of Neurochemistry, Volume 97, Number 6, June 2006 , pp. 1690-1699(10)

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Abstract:

In the last 50 years, an enormous amount of progress has been made in dissecting the etiology of hereditary neurodegenerative diseases, including the dementias, the parkinsonisms, the ataxias and the motor-neuron diseases. In addition, these genetic findings are beginning to provide insights into the pathogeneses of the sporadic forms of the diseases. Through animal and cellular modeling studies we are beginning to gain insights into the pathogenic pathways to disease. This mechanistic understanding is now leading to therapeutic strategies based on this new understanding. As yet, however, no mechanistic therapies are in use in the clinic.

Keywords: ataxia; dementia; parkinsonism; polyglutamine; α-synuclein; tau

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1471-4159.2006.03979.x

Affiliations: 1: Department of Biochemistry, Molecular Biology, Biophysics, Department of Laboratory Medicine and Pathology and the Institute of Human Genetics, The University of Minnesota, Minneapolis, USA

Publication date: 2006-06-01