Turner MR, Al-Chalabi A, Chio A, et al. Genetic screening in sporadic ALS and FTD. J Neurol Neurosurg Psychiatry 2017;88:1042-4.Turner MR, Al-Chalabi A, Chio A, et al. Genetic screening in sporadic ALS and FT
slices are optimal for shorter term drug treatments, slices cultured at the ALI could be a preferred platform for longer term drug testing. Scaling up this approach using many patient cell lines for more comprehensive drug screening applications would require further technical efforts and automation fo...
High-content RNAi screening identifies the type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity Hum. Mol. Genet., 21 (2012), pp. 4845-4856, 10.1093/hmg/dds321 View in ScopusGoogle Scholar Kinoshita et al., 2009 Y. Kinoshita, H. Ito, A. Hirano, K...
After compiling all data, two researchers assigned independently a status to each RNAi line (no effect, enhancer or suppressor) as compared to a cross of the screening stock to the RNAi w1118 background. Only whenever a gene was represented by at least two RNAi lines showing a similar ...
[200]. Small compounds targeting the peculiar G-quadruplex structure of C9ORF72 repeats have successfully used to reduced RNA foci burden and the levels of DPRs in patient iPSC-derived motor and cortical neurons [201]. Likewise to TDP-43 pathology modifiers, the screening for repressors of C9...
Identification of a small molecule inhibitor of importin beta mediated nuclear import by confocal on-bead screening of tagged one-bead one-compound libraries. ACS Chem Biol. 2010;5:967–79. 97. Nag N, Sasidharan S, Uversky VN, Saudagar P, Tripathi T. Phase separa- tion of FG-nucleoporins...
Variants of the yeast protein disaggregase Hsp104 were also recently identified by systematic screening and shown to rescue TDP-43 toxicity [229]. Compared to small molecule- or RNA-based therapeutics, the neuronal delivery of the protein or peptide-based therapies is more challenging. Still, ...
[200]. Small compounds targeting the peculiar G-quadruplex structure of C9ORF72 repeats have successfully used to reduced RNA foci burden and the levels of DPRs in patient iPSC-derived motor and cortical neurons [201]. Likewise to TDP-43 pathology modifiers, the screening for repressors of C9...
193Accesses Synonyms Behavioural variant frontotemporal dementia; Frontal dementia; Semantic Dementia; Progressive non-fluent aphasia; Pick disease Definition Frontotemporal dementia is an insidious neurodegenerative disease characterised by progressive deficits in behaviour and cognition. Three main clinical entities...
Ferrari R, Mok K, Moreno JH, Cosentino S, Goldman J, Pietrini P, Mayeux R, Tierney MC, Kapogiannis D, Jicha GA, Murrell JR, Ghetti B, Wassermann EM, Grafman J, Hardy J, Huey ED, Momeni P: Screening for C9ORF72 repeat expansion in FTLD. Neurobiol Aging. 2012, 33: 1850.e1-1850...