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The Parkinson’s Disease-Associated H50Q Mutation Accelerates α‑Synuclein Aggregation in Vitro

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journal contribution
posted on 2013-10-08, 00:00 authored by Dhiman Ghosh, Mrityunjoy Mondal, Ganesh M. Mohite, Pradeep K. Singh, Priyatosh Ranjan, A. Anoop, Saikat Ghosh, Narendra Nath Jha, Ashutosh Kumar, Samir K. Maji
α-Synuclein (α-Syn) aggregation is directly linked with Parkinson’s disease (PD) pathogenesis. Here, we analyzed the aggregation of newly discovered α-Syn missense mutant H50Q in vitro and found that this mutation significantly accelerates the aggregation and amyloid formation of α-Syn. This mutation, however, did not alter the overall secondary structure as suggested by two-dimensional nuclear magnetic resonance and circular dichroism spectroscopy. The initial oligomerization study by cross-linking and chromatographic techniques suggested that this mutant oligomerizes to an extent similar to that of the wild-type α-Syn protein. Understanding the aggregation mechanism of this H50Q mutant may help to establish the aggregation and phenotypic relationship of this novel mutant in PD.

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