@article{oai:soar-ir.repo.nii.ac.jp:00021774,
 author = {Verheijen, Bert M. and Oyanagi, Kiyomitsu and van, Leeuwen  Fred W.},
 journal = {FRONTIERS IN NEUROLOGY},
 month = {Mar},
 note = {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., Article, FRONTIERS IN NEUROLOGY.9:173(2018)},
 title = {Dysfunction of Protein Quality control in Parkinsonism-Dementia complex of Guam},
 volume = {9},
 year = {2018}
}