Literature DB >> 24047453

The Parkinson's disease-associated H50Q mutation accelerates α-Synuclein aggregation in vitro.

Dhiman Ghosh1, Mrityunjoy Mondal, Ganesh M Mohite, Pradeep K Singh, Priyatosh Ranjan, A Anoop, Saikat Ghosh, Narendra Nath Jha, Ashutosh Kumar, Samir K Maji.   

Abstract

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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Year:  2013        PMID: 24047453     DOI: 10.1021/bi400999d

Source DB:  PubMed          Journal:  Biochemistry        ISSN: 0006-2960            Impact factor:   3.162


  65 in total

1.  Coupling of the non-amyloid-component (NAC) domain and the KTK(E/Q)GV repeats stabilize the α-synuclein fibrils.

Authors:  Liang Xu; Ruth Nussinov; Buyong Ma
Journal:  Eur J Med Chem       Date:  2016-01-25       Impact factor: 6.514

2.  A reduced dimensionality NMR pulse sequence and an efficient protocol for unambiguous assignment in intrinsically disordered proteins.

Authors:  Jithender G Reddy; Ramakrishna V Hosur
Journal:  J Biomol NMR       Date:  2014-05-23       Impact factor: 2.835

3.  Glycosaminoglycans have variable effects on α-synuclein aggregation and differentially affect the activities of the resulting amyloid fibrils.

Authors:  Surabhi Mehra; Dhiman Ghosh; Rakesh Kumar; Mrityunjoy Mondal; Laxmikant G Gadhe; Subhadeep Das; Arunagiri Anoop; Narendra N Jha; Reeba S Jacob; Debdeep Chatterjee; Soumik Ray; Nitu Singh; Ashutosh Kumar; Samir K Maji
Journal:  J Biol Chem       Date:  2018-06-29       Impact factor: 5.157

4.  Familial Parkinson disease-associated mutations alter the site-specific microenvironment and dynamics of α-synuclein.

Authors:  Shruti Sahay; Dhiman Ghosh; Saumya Dwivedi; Arunagiri Anoop; Ganesh Maruti Mohite; Mamata Kombrabail; Guruswamy Krishnamoorthy; Samir K Maji
Journal:  J Biol Chem       Date:  2015-01-29       Impact factor: 5.157

5.  Familial Mutations May Switch Conformational Preferences in α-Synuclein Fibrils.

Authors:  Liang Xu; Buyong Ma; Ruth Nussinov; Damien Thompson
Journal:  ACS Chem Neurosci       Date:  2017-01-27       Impact factor: 4.418

6.  Comparison of the in vivo induction and transmission of α-synuclein pathology by mutant α-synuclein fibril seeds in transgenic mice.

Authors:  Nicola J Rutherford; Jess-Karan S Dhillon; Cara J Riffe; Jasie K Howard; Mieu Brooks; Benoit I Giasson
Journal:  Hum Mol Genet       Date:  2017-12-15       Impact factor: 6.150

7.  Structural features of α-synuclein amyloid fibrils revealed by Raman spectroscopy.

Authors:  Jessica D Flynn; Ryan P McGlinchey; Robert L Walker; Jennifer C Lee
Journal:  J Biol Chem       Date:  2017-11-30       Impact factor: 5.157

8.  The A53E α-synuclein pathological mutation demonstrates reduced aggregation propensity in vitro and in cell culture.

Authors:  Nicola J Rutherford; Benoit I Giasson
Journal:  Neurosci Lett       Date:  2015-04-17       Impact factor: 3.046

9.  Role of Parkinson's Disease-Linked Mutations and N-Terminal Acetylation on the Oligomerization of α-Synuclein Induced by 3,4-Dihydroxyphenylacetaldehyde.

Authors:  Vanderlei de Araújo Lima; Lucas Alex do Nascimento; David Eliezer; Cristian Follmer
Journal:  ACS Chem Neurosci       Date:  2018-11-05       Impact factor: 4.418

10.  The H50Q mutation enhances α-synuclein aggregation, secretion, and toxicity.

Authors:  Ossama Khalaf; Bruno Fauvet; Abid Oueslati; Igor Dikiy; Anne-Laure Mahul-Mellier; Francesco Simone Ruggeri; Martial K Mbefo; Filip Vercruysse; Giovanni Dietler; Seung-Jae Lee; David Eliezer; Hilal A Lashuel
Journal:  J Biol Chem       Date:  2014-06-16       Impact factor: 5.157

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