Literature DB >> 27374978

MAPT haplotype diversity in multiple system atrophy.

Catherine Labbé1, Michael G Heckman2, Oswaldo Lorenzo-Betancor1, Melissa E Murray1, Kotaro Ogaki1, Alexandra I Soto-Ortolaza1, Ronald L Walton1, Shinsuke Fujioka1, Shunsuke Koga1, Ryan J Uitti3, Jay A van Gerpen3, Ronald C Petersen4, Neill R Graff-Radford3, Steven G Younkin1, Bradley F Boeve4, William P Cheshire3, Phillip A Low4, Paola Sandroni4, Elizabeth A Coon4, Wolfgang Singer4, Zbigniew K Wszolek3, Dennis W Dickson1, Owen A Ross5.   

Abstract

INTRODUCTION: Multiple system atrophy (MSA) is a rare progressive neurodegenerative disorder. MSA was originally considered exclusively sporadic but reports of association with genes such as SNCA, COQ2 and LRRK2 have demonstrated that there is a genetic contribution to the disease. MAPT has been associated with several neurodegenerative diseases and we previously reported a protective association of the MAPT H2 haplotype with MSA in 61 pathologically confirmed cases.
METHODS: In the present study, we assessed the full MAPT haplotype diversity in MSA patients using six MAPT tagging SNPs. We genotyped a total of 127 pathologically confirmed MSA cases, 86 patients with clinically diagnosed MSA and 1312 controls.
RESULTS: We identified four significant association signals in our pathologically confirmed cases, two from the protective haplotypes H2 (MSA:16.2%, CONTROLS: 22.7%, p = 0.024) and H1E (MSA:3.0%, CONTROLS: 9.0%, p = 0.014), and two from the rare risk haplotypes H1x (MSA:3.7%, CONTROLS: 1.3%, p = 0.030) and H1J (MSA:3.0%, CONTROLS: 0.9%, p = 0.021). We evaluated the association of MSA subtypes with the common protective H2 haplotype and found a significant difference with controls for MSA patients with some degree of MSA-C (MSA-C or MSA-mixed), for whom H2 occurred in only 8.6% of patients in our pathologically confirmed series (P < 0.0001).
CONCLUSIONS: Our findings provide further evidence that MAPT variation is associated with risk of MSA. Interestingly, our results suggest a greater effect size in the MSA-C compared to MSA-P for H2. Additional genetic studies in larger pathologically confirmed MSA series and meta-analytic studies will be needed to fully assess the role of MAPT and other genes in MSA.
Copyright © 2016 Elsevier Ltd. All rights reserved.

Entities:  

Keywords:  Association study; Genetics; MAPT; Multiple system atrophy

Mesh:

Substances:

Year:  2016        PMID: 27374978      PMCID: PMC5007155          DOI: 10.1016/j.parkreldis.2016.06.010

Source DB:  PubMed          Journal:  Parkinsonism Relat Disord        ISSN: 1353-8020            Impact factor:   4.891


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