Distinct α-Synuclein strains and implications for heterogeneity among α-Synucleinopathies

NEUROBIOLOGY OF DISEASE

卷:109

Review

Peng, Chao ; Gathagan, Ronald J. ; Lee, Virginia M. -Y.

关键词: alpha-Synuclein strains;    Protein aggregates;    alpha-Synucleinopathy;    Multiple system atrophy;    Parkinson's disease;    Dementia with Lewy body;   

DOI  :  10.1016/j.nbd.2017.07.018

来源: Elsevier

【 摘 要 】

The deposition of misfolded beta-sheet enriched amyloid protein is a shared feature of many neurodegenerative diseases. Recent studies demonstrated the existence of conformationally diverse strains as a common property for multiple amyloidogenic proteins including alpha-Synuclein (alpha-Syn). alpha-Syn is misfolded and aggregated in a group of neurodegenerative diseases collectively known as alpha-Synucleinopathies, which include Parkinson's disease (PD), dementia with Lewy body, multiple system atrophy and also a subset of Alzheimer's disease patients with concomitant PD-like Lewy bodies and neurites. While sharing the same pathological protein, different alpha-Synucleinopathies demonstrate distinct clinical and pathological phenotypes, which could result from the existence of diverse pathological alpha-Syn strains in patients. In this review, we summarized the characteristics of different a-Synucleinopathies and alpha-Syn strains generated with recombinant alpha-Syn monomers. We also make predictions of a-Syn strains that could potentially exist in patients based on the knowledge from other amyloid proteins and the clinical and pathological features of different alpha-Synucleinopathies. (C) 2017 Elsevier Inc. All rights reserved.

【 授权许可】

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