Journal article

DYSREGULATION OF SYNAPTIC PROTEINS, DENDRITIC SPINE ABNORMALITIES AND PATHOLOGICAL PLASTICITY OF SYNAPSES AS EXPERIENCE-DEPENDENT MEDIATORS OF COGNITIVE AND PSYCHIATRIC SYMPTOMS IN HUNTINGTON'S DISEASE

J Nithianantharajah, AJ Hannan

NEUROSCIENCE | PERGAMON-ELSEVIER SCIENCE LTD | Published : 2013

Abstract

Huntington's disease (HD) is an autosomal dominant tandem repeat expansion disorder involving cognitive, psychiatric and motor symptoms. The expanded trinucleotide (CAG) repeat leads to an extended polyglutamine tract in the huntingtin protein and a subsequent cascade of molecular and cellular pathogenesis. One of the key features of neuropathology, which has been shown to precede the eventual loss of neurons in the cerebral cortex, striatum and other areas, are changes to synapses, including the dendritic protrusions known as spines. In this review we will focus on synapse and spine pathology in HD, including molecular and experience-dependent aspects of pathogenesis. Dendritic spine pathol..

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