Inherited neurodegenerative diseases and transgenic models

Brain Pathology
D L PriceS S Sisodia

Abstract

In recent years, the identification of mutations in specific genes in several inherited neurodegenerative disorders, combined with advances in the field of transgenic methods, has provided neuroscientists and neuropathologists with information and strategies to develop transgenic (Tg) models to study human diseases. These approaches have proved to be extraordinarily useful in modeling familial forms of amyotrophic lateral sclerosis (FALS) and Alzheimer's disease (FAD) and the spectrum of triplet-repeat disorders. Investigations of these models have begun to provide new insights into the roles of disease-specific mutant proteins and the pathogenic mechanisms of disease as well as opportunities to test therapeutic interventions.

Citations

Sep 12, 2003·BMC Molecular Biology·Sonia RochaNeil D Perkins
Aug 26, 2011·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Michele A KliemNicholas M Boulis
Dec 4, 2004·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Catherine DesrumauxLaurent Lagrost

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