Neurodegenerative a-synucleinopathies and tauopathies

NEURODEGENERATIVE a-SYNUCLEINOPATHIES AND TAUOPATHIES 745 NEUROTRANSMITTERS AND DISORDERS OF THE BASAL GANGLIA 761 NEUROBIOLOGY OF ALZHEIMER S DISEASE 781 MOLECULAR BASIS OF PRION DISEASES 791... 

Lewy bodies, neurofibrillary lesions and Pick bodies are intracellular filamentous inclusions. It is now well established that Lewy bodies are made of the protein a-synuclein and both neurofibrillary lesions and Pick bodies of the microtubule-associated protein tau. Mutations in the a-synuclein gene or an increase in its copy number cause autosomal-dominantly inherited forms of Parkinson s disease and dementia with Lewy bodies. Mutations in the tau gene cause a familial form of frontotemporal dementia. Here we review the evidence implicating a-synuclein and tau in these inherited and a number of sporadic neurodegenerative diseases. Collectively, a-synucleinopathies and tauopathies account for the vast majority of cases of late-onset neurodegenerative disease (Tables 45-1 and 45-2). 

Goedert M. Filamentous nerve cell inclusions in neurodegenerative diseases tauopathies and a-synucleinopathies. Philos Trans R Soc Lond B 1999 354 1101-1118. 

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