Disordered proteins in dementia.
Aggregates of dysfunctional proteins and peptides in or between brain neurons are key neuropathological features of dementia and are believed to directly cause or substantially contribute to the development of these diseases. Fundamental parts of the mechanisms underlying the dysregulation of proteins in Alzheimer's disease, frontotemporal dementia, prion diseases and other dementing disorders are now well characterized, mainly due to the discovery of genes causing dominantly inherited disease forms (Table 1). As of today, no efficient pharmacotherapies are available, but new insights into the underlying molecular mechanisms are providing strategies to prevent or even cure these devastating disorders.
Ingelsson
Martin
M
Harvard Medical School, Massachusetts General Hospital, Charlestown 02129, USA.
Hyman
Bradley T
BT
D016229:Amyloid beta-Peptides / Q000235:genetics / Q000378:metabolism; D016564:Amyloid beta-Protein Precursor / Q000235:genetics / Q000378:metabolism; D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D003704:Dementia / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D016631:Lewy Bodies / Q000235:genetics / Q000378:metabolism; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism; D011328:Prions / Q000235:genetics / Q000378:metabolism; D051843:Synucleins; D016875:tau Proteins / Q000235:genetics / Q000378:metabolism
D016229:Amyloid beta-Peptides; D016564:Amyloid beta-Protein Precursor; D009419:Nerve Tissue Proteins; D011328:Prions; D051843:Synucleins; D016875:tau Proteins