Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.

doi:10.1016/j.neuron.2015.11.018

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Article

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.

Neuron - 2 Dec 2015

Peters Owen M, Cabrera Gabriela Toro, Tran Helene, Gendron Tania F, McKeon Jeanne E, Metterville Jake, Weiss Alexandra, Wightman Nicholas, Salameh Johnny, Kim Juyhun, Sun Huaming, Boylan Kevin B, Dickson Dennis, Kennedy Zack, Lin Ziqiang, Zhang Yong-Jie, Daughrity Lillian, Jung Chris, Gao Fen-Biao, Sapp Peter C, Horvitz H Robert, Bosco Daryl A, Brown Solange P, de Jong Pieter, Petrucelli Leonard, Mueller Chris, Brown Robert H

Abstract excerpt

A non-coding hexanucleotide repeat expansion in the C9ORF72 gene is the most common mutation associated with familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). To investigate the pathological role of C9ORF72 in these diseases, we generated a line of mice carrying a bacterial artificial chromosome containing exons 1 to 6 of the human C9ORF72 gene with approximately 500 repeats of the...

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PMID: 26637797
DOI: 10.1016/j.neuron.2015.11.018
PMCID: PMC4828340

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