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

Acta Neuropathologica Communications

Volumn 2, Issue 1, 2014, Pages

  Hukema, Renate K ^a,e   Riemslagh, Fréderike W ^a   Melhem, Shamiram ^a   van der Linde, Herma C ^a   Severijnen, Lies Anne W F M ^a   Edbauer, Dieter ^b   Maas, Alex ^a   Charlet Berguerand, Nicolas ^c   Willemsen, Rob ^a   van Swieten, John C ^a,d  

^a ERASMUS MEDICAL CENTER   (Netherlands)

^b GERMAN CENTER FOR NEURODEGENERATIVE DISEASES DZNE   (Germany)

^c UNIVERSITÉ DE STRASBOURG   (France)

^d Department of Cell Biology ^*  (Netherlands)

^e Rotterdam University   (Netherlands)

Author keywords

ALS; C9orf72; FTD; GGGGCC repeat; Inducible mouse model; RNA gain of function

Indexed keywords

C9ORF72 PROTEIN, HUMAN; PROTEIN; REPETITIVE DNA; UBIQUITIN;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL; BRAIN; CELL INCLUSION; DISEASE MODEL; FRONTOTEMPORAL DEMENTIA; GENETICS; HUMAN; METABOLISM; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; BRAIN; DISEASE MODELS, ANIMAL; DNA REPEAT EXPANSION; FRONTOTEMPORAL DEMENTIA; HUMANS; INCLUSION BODIES; MICE, TRANSGENIC; PROTEINS; UBIQUITIN;

EID: 84964697584     PISSN: None     EISSN: 20515960     Source Type: Journal    
DOI: 10.1186/s40478-014-0166-y     Document Type: Letter

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