A new inducible transgenic mouse model for C9orf72-associated GGGGCC repeat expansion supports a gain-of-function mechanism in C9orf72-associated ALS and FTD

A new inducible transgenic mouse model for C9orf72-associated GGGGCC repeat expansion supports a gain-of-function mechanism in C9orf72-associated ALS and FTD

Keywords: C9orf72, GGGGCC repeat, ALS, FTD, Inducible mouse model, RNA gain-of-function

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Veröffentlicht in:Acta neuropathologica communications 2014-12, Vol.2, p.166-166
Hauptverfasser: Hukema, Renate K, Riemslagh, Fréderike W, Melhem, Shamiram, van der Linde, Herma C, Severijnen, Lies-Anne Wfm, Edbauer, Dieter, Maas, Alex, Charlet-Berguerand, Nicolas, Willemsen, Rob, van Swieten, John C
Format: Artikel
Sprache:eng
Schlagworte:
Amyotrophic Lateral Sclerosis - genetics
Amyotrophic Lateral Sclerosis - metabolism
Analysis
Animals
Brain - metabolism
C9orf72 Protein
Dementia
Development and progression
Disease Models, Animal
DNA Repeat Expansion
Frontotemporal Dementia - genetics
Frontotemporal Dementia - metabolism
Genetic aspects
Humans
Inclusion Bodies - metabolism
Letter to the Editor
Mice, Transgenic
Nervous system diseases
Proteins - genetics
Proteins - metabolism
RNA
Ubiquitin - metabolism
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Zusammenfassung:Keywords: C9orf72, GGGGCC repeat, ALS, FTD, Inducible mouse model, RNA gain-of-function
ISSN:2051-5960
2051-5960
DOI:10.1186/s40478-014-0166-y