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Current Neuropharmacology


ISSN (Print): 1570-159X
ISSN (Online): 1875-6190

Recent Advances in the Molecular Pathogenesis of Dystonia-Plus Syndromes and Heredodegenerative Dystonias

Author(s): Catharina Casper, Eirini Kalliolia and Thomas T. Warner

Volume 11, Issue 1, 2013

Page: [30 - 40] Pages: 11

DOI: 10.2174/1570159X11311010005

Price: $65


The majority of studies investigating the molecular pathogenesis and cell biology underlying dystonia have been performed in individuals with primary dystonia. This includes monogenic forms such as DYT1and DYT6 dystonia, and primary focal dystonia which is likely to be multifactorial in origin. In recent years there has been renewed interest in non-primary forms of dystonia including the dystonia-plus syndromes and heredodegenerative disorders. These are caused by a variety of genetic mutations and their study has contributed to our understanding of the neuronal dysfunction that leads to dystonia These findings have reinforced themes identified from study of primary dystonia including abnormal dopaminergic signalling, cellular trafficking and mitochondrial function. In this review we highlight recent advances in the understanding of the dystonia-plus syndromes and heredodegenerative dystonias.

Keywords: Dystonia- plus syndrome, Secondary dystonia, Molecular pathogenesis, Neurodegeneration, Mitochondria

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