Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Abstract Frontotemporal lobar degeneration (FTLD) is heterogeneous in clinical presentation, neuropathological characteristics and genetics. An expanded GGGGCC hexanucleotide repeat in C9ORF72 is the most common genetic cause of both FTLD and motor neuron disease (MND). Dipeptide repeat polymers (DP...

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Bibliographic Details
Main Authors: Nobutaka Sakae, Kevin F. Bieniek, Yong-Jie Zhang, Kelly Ross, Tania F. Gendron, Melissa E. Murray, Rosa Rademakers, Leonard Petrucelli, Dennis W. Dickson
Format: Article
Language:English
Published: BMC 2018-07-01
Series:Acta Neuropathologica Communications
Subjects:
C9ORF72
Dipeptide repeat polymers (DPR)
Poly-GR
Neurodegeneration
Dimethylarginine
Online Access:http://link.springer.com/article/10.1186/s40478-018-0564-7

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