Neurodegenerative diseases sporadic

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). 

Benhar M, Forrester MT, Stamler JJ (2006) Nitrosative stress in the ER a new role for S-nitrosylation in neurodegenerative diseases. ACS Chem Biol 1 355-358 BenMoyal-Segal L, Soreq H (2006) Gene-environment interactions in sporadic Parkinson s disease. J Neurochem 97 1740-1755... 

Alterations in UPS fnnction have been implicated in the pathogenesis of a variety of sporadic and familial neurodegenerative diseases including Parkinson disease, Alzheimer disease, polyglutamine repeat diseases, and ALS [63-65]. Mizuno et al. [66] called VCP "vacuole-creating protein" and demonstrated that VCP was observed in ubiquitin-positive intraneuronal inclusions in both motor neuron disease with dementia, and ballooned neurons in Creutzfeldt-Jakob disease. In Alzheimer disease, VCP has been found in dystrophic neurites while... 

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