Western Pacific ALS-PDC: a prototypical neurodegenerative disorder linked to DNA damage and aberrant proteogenesis?
نویسندگان
چکیده
The Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS-PDC) has been described as a Rosetta Stone that bears the essential clue to understanding the etiopathogenesis of related neurodegenerative diseases. The three clinical forms (ALS, atypical parkinsonism with dementia, and dementia alone) have a single pathology (polyproteinopathy, notably tauopathy), just as the Rosetta Stone is inscribed with three distinct scripts bearing a common message. As recently discussed (Kisby and Spencer, 2011), studies of ALS-PDC in the three geographically separate and genetically distinct island populations (Chamorros on Guam; Japanese in Honshu Island’s Kii Peninsula; and Papuan New Guineans in Irian Jaya, Indonesia) show:
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عنوان ژورنال:
دوره 3 شماره
صفحات -
تاریخ انتشار 2012