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1-3 of 3
Keywords: FUS
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Journal Articles
In collection:
Neurodegenerative Disorders
Martin Crivello, Marion C. Hogg, Elisabeth Jirström, Luise Halang, Ina Woods, Megan Rayner, Karen S. Coughlan, Sebastian A. Lewandowski, Jochen H. M. Prehn
Journal:
Disease Models & Mechanisms
Dis Model Mech (2019) 12 (8): dmm040238.
Published: 13 August 2019
... stages of disease progression remains to be established in other ALS models. Here, we assessed vascular integrity in vivo throughout different disease stages, and investigated whether ANG treatment reverses vascular regression and prolongs motor neuron survival in the FUS (1-359) mouse model of ALS...
Includes: Supplementary data
Journal Articles
Jessica Lenzi, Riccardo De Santis, Valeria de Turris, Mariangela Morlando, Pietro Laneve, Andrea Calvo, Virginia Caliendo, Adriano Chiò, Alessandro Rosa, Irene Bozzoni
Journal:
Disease Models & Mechanisms
Dis Model Mech (2015) 8 (7): 755–766.
Published: 1 July 2015
... cases for which no suitable model systems are available. Here, we have taken advantage of in vitro iPSCs derived from patients affected by amyotrophic lateral sclerosis (ALS) and carrying mutations in the RNA-binding protein FUS to study the cellular behavior of the mutant proteins in the appropriate...
Includes: Supplementary data
Journal Articles
Journal:
Disease Models & Mechanisms
Dis Model Mech (2014) 7 (10): 1175–1184.
Published: 1 October 2014
...-43, FUS and α-synuclein, as well as missense mutant versions of these proteins that cause neurodegenerative disease. Potentiated Hsp104 variants also rescue toxicity and aggregation of TAF15 but not EWSR1, two RNA-binding proteins with a prion-like domain that are connected with the development...