Corticobasal degeneration

They include progressive supranuclear palsy, corticobasal degeneration and Pick s disease 753... 

TABLE 45-2 Tau diseases Alzheimer s disease Down s syndrome Progressive supranuclear palsy Corticobasal degeneration Pick s disease Argyrophilic grain disease Tangle-only dementia... 

The study of FTDP-17 has established that dysfunction or misregulation of tau protein can cause neurodegeneration and dementia. It follows that tau protein is probably also of central importance in the pathogenesis of diseases, such as Alzheimer s disease, progressive supranuclear palsy, corticobasal degeneration and Pick s disease. This is further underlined by the fact that the aforementioned diseases are partially or completely phenocopied by cases of FTDP-17. 

Tau pathology in AD is circumscribed to neurons, while in other tauopathies, such as corticobasal degeneration, PSP and familial multiple system tauopathy with presenile dementia, both nerve cells and ghal cells are affected (210,211). PINl binds hyperphosphorylated tau, resulting in depletion of soluble PINl in AD brains. 

Dickson DW, Bergeron C, Chin SS, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. Neuro-pathol Exp Neurol. 2002 61 935-946. 

CasteUani, R., Smith, M.A., Richey, P.L., Kalaria, R., Gambetti, P. and Perry, G. 1995. Evidence for oxidative stress in Pick disease and corticobasal degeneration. Brain Res. 696 268-271 Cervantes, S., Gonzalez-Duarte, R. and Marfany, G. 2001. Homodimerization of presemlin N-terminal fragments is affected by mutations linked to Alzheimer s disease. FEBS Lett. 505 81-86... 

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