Dysfunction of Protein Quality Control in Parkinsonism–Dementia Complex of Guam

Guam parkinsonism-dementia complex (G-PDC) is an enigmatic neurodegenerative disease that is endemic to the Pacific island of Guam. G-PDC patients are clinically characterized by progressive cognitive impairment and parkinsonism. Neuropathologically, G-PDC is characterized by abundant neurofibrillar...

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Published in:Frontiers in neurology Vol. 9; p. 173
Main Authors: Verheijen, Bert M., Oyanagi, Kiyomitsu, van Leeuwen, Fred W.
Format: Journal Article
Language:English
Published: Switzerland Frontiers Media S.A 20.03.2018
Subjects:
Guam parkinsonism–dementia complex
mutant ubiquitin
neurofibrillary tangles
Neuroscience
protein aggregation
protein quality control
tau
mutant ubiquitin
Guam parkinsonism–dementia complex
protein aggregation
neurofibrillary tangles
protein quality control
tau
43-kDa TAR DNA-binding protein
neuropathology
ISSN:1664-2295, 1664-2295
Online Access:Get full text
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Summary:Guam parkinsonism-dementia complex (G-PDC) is an enigmatic neurodegenerative disease that is endemic to the Pacific island of Guam. G-PDC patients are clinically characterized by progressive cognitive impairment and parkinsonism. Neuropathologically, G-PDC is characterized by abundant neurofibrillary tangles, which are composed of hyperphosphorylated tau, marked deposition of 43-kDa TAR DNA-binding protein, and neuronal loss. Although both genetic and environmental factors have been implicated, the etiology and pathogenesis of G-PDC remain unknown. Recent neuropathological studies have provided new clues about the pathomechanisms involved in G-PDC. For example, deposition of abnormal components of the protein quality control system in brains of G-PDC patients indicates a role for proteostasis imbalance in the disease. This opens up promising avenues for new research on G-PDC and could have important implications for the study of other neurodegenerative disorders.
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Edited by: Cintia Roodveldt, Centro Andaluz de Biología Molecular y Medicina Regenerativa (CABIMER), Spain
Reviewed by: Arianna Bellucci, University of Brescia, Italy; Carlos M. Opazo, University of Melbourne, Australia
Specialty section: This article was submitted to Neurodegeneration, a section of the journal Frontiers in Neurology
ISSN:1664-2295
1664-2295
DOI:10.3389/fneur.2018.00173