Protein aggregate spreading in neurodegenerative diseases: Problems and perspectives
doi:10.1016/j.neures.2011.05.008 | How to Cite or Link Using DOI Permissions & Reprints
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Seung-Jae Leea, c, , , Hee-Sun Lima, c, Eliezer Masliahd and He-Jin Leeb, c
a Department of Biomedical Science and Technology, Konkuk University, Seoul 143-701, Republic of Korea
b Department of Anatomy School of Medicine, Konkuk University, Seoul 143-701, Republic of Korea
c Institute of Biomedical Science and Technology, Konkuk University, Seoul 143-701, Republic of Korea
d Department of Neurosciences and Pathology, School of Medicine, University of California San Diego, La Jolla, CA 92093-0624, USA
Received 4 March 2011; revised 13 May 2011; accepted 16 May 2011. Available online 20 May 2011.
Progressive accumulation of specific protein aggregates is a defining feature of many major neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, fronto-temporal dementia, Huntington's disease, and Creutzfeldt–Jakob disease (CJD). Findings from several recent studies have suggested that aggregation-prone proteins, such as tau, a-synuclein, polyglutamine-containing proteins, and amyloid-ß, can spread to other cells and brain regions, a phenomenon considered unique to prion disorders, such as CJD and bovine spongiform encephalopathy. Cell-to-cell propagation of protein aggregates may be the general underlying principle for progressive deterioration of neurodegenerative diseases. This may also have significant implications in cell replacement therapies, as evidenced by the propagation of a-synuclein aggregates from host to grafted cells in long-term transplants in Parkinson's patients. Here, we review recent progress in protein aggregate propagation in experimental model systems and discuss outstanding questions and future perspectives. Understanding the mechanisms of this pathological spreading may open the way to unique opportunities for development of diagnostic techniques and novel therapies for protein misfolding-associated neurodegenerative diseases.
Keywords: Protein aggregation; Alzheimer's disease; Parkinson's disease; Huntington's disease; Prion
Abbreviations: CJD, Creutzfeldt–Jakob disease; AD, Alzheimer's disease; PD, Parkinson's disease; HD, Huntington's disease; Aß, amyloid-ß; polyQ, polyglutamine; CNS, central nervous system; NFTs, neurofibrillary tangles; NTs, neuropil threads; APP, amyloid precursor protein
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