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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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Principais autores: Nobutaka Sakae, Kevin F. Bieniek, Yong-Jie Zhang, Kelly Ross, Tania F. Gendron, Melissa E. Murray, Rosa Rademakers, Leonard Petrucelli, Dennis W. Dickson
Format: Artigo
Sprog:Inglês
Udgivet: BMC 2018-07-01
Serier:Acta Neuropathologica Communications
Fag:
C9ORF72
Dipeptide repeat polymers (DPR)
Poly-GR
Neurodegeneration
Dimethylarginine
Online adgang:http://link.springer.com/article/10.1186/s40478-018-0564-7
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