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Acta Neuropathologica, ISSN 0001-6322, 06/2017, Volume 133, Issue 6, pp. 923 - 931
Abnormal accumulation of TDP-43 into cytoplasmic or nuclear inclusions with accompanying nuclear clearance, a common pathology initially identified in... 
Hippocampal sclerosis | ALS/FTD | Alzheimer’s disease | Neurodegeneration | TDP-43 proteinopathy | Cryptic exon | cryptic exon | FTD | ALS | neurodegeneration
Journal Article
Frontiers in Neurology, ISSN 1664-2295, 03/2019, Volume 10
Single time point positron emission tomography (PET) studies of patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD), have... 
aphasia | MRI | cortical area | ALS-FTD | cortical thickness | PET
Journal Article
Journal Article
Journal of Neuroscience, ISSN 0270-6474, 08/2018, Volume 38, Issue 35, pp. 7741 - 7752
The arginine-rich dipeptide repeats (DPRs) are highly toxic products from the C9orf72 repeat expansion mutations, which are the most common causes of familial... 
ALS | Selective neurotoxicity | C9orf72 dipeptide repeats | Excitotoxicity | FTD | Cell-autonomous | ALS/FTD | ALZHEIMERS-DISEASE | AMYOTROPHIC-LATERAL-SCLEROSIS | BAC TRANSGENIC MICE | MOTOR-NEURON | NEUROSCIENCES | TRANSMITTER RELEASE | selective neurotoxicity | EXPANSION | cell-autonomous | excitotoxicity | HEXANUCLEOTIDE REPEAT | NUCLEOCYTOPLASMIC TRANSPORT | RNA FOCI
Journal Article
by Gendron, Tania F and Chew, Jeannie and Stankowski, Jeannette N and Hayes, Lindsey R and Zhang, Yong-Jie and Prudencio, Mercedes and Carlomagno, Yari and Daughrity, Lillian M and Jansen-West, Karen and Perkerson, Emilie A and O'Raw, Aliesha and Cook, Casey and Pregent, Luc and Belzil, Veronique and Van Blitterswijk, Marka and Tabassian, Lilia J and Lee, Chris W and Yue, Mei and Tong, Jimei and Song, Yuping and Castanedes-Casey, Monica and Rousseau, Linda and Phillips, Virginia and Dickson, Dennis W and Rademakers, Rosa and Fryer, John D and Rush, Beth K and Pedraza, Otto and Caputo, Ana M and Desaro, Pamela and Palmucci, Carla and Robertson, Amelia and Heckman, Michael G and Diehl, Nancy N and Wiggs, Edythe and Tierney, Michael and Braun, Laura and Farren, Jennifer and Lacomis, David and Ladha, Shafeeq and Fournier, Christina N and McCluskey, Leo F and Elman, Lauren B and Toledo, Jon B and McBride, Jennifer D and Tiloca, Cinzia and Morelli, Claudia and Poletti, Barbara and Solca, Federica and Prelle, Alessandro and Wuu, Joanne and Jockel-Balsarotti, Jennifer and Rigo, Frank and Ambrose, Christine and Datta, Abhishek and Yang, Weixing and Raitcheva, Denitza and Antognetti, Giovanna and McCampbell, Alexander and Van Swieten, John C and Miller, Bruce L and Boxer, Adam L and Brown, Robert H and Bowser, Robert and Miller, Timothy M and Trojanowski, John Q and Grossman, Murray and Berry, James D and Hu, William T and Ratti, Antonia and Traynor, Bryan J and Disney, Matthew D and Benatar, Michael and Silani, Vincenzo and Glass, Jonathan D and Floeter, Mary Kay and Rothstein, Jeffrey D and Boylan, Kevin B and Petrucelli, Leonard
Science Translational Medicine, ISSN 1946-6234, 03/2017, Volume 9, Issue 383, pp. eaai7866 - eaai7866
Journal Article
Brain, ISSN 0006-8950, 2012, Volume 135, Issue 3, pp. 709 - 722
Journal Article
Acta Neuropathologica, ISSN 0001-6322, 3/2012, Volume 123, Issue 3, pp. 409 - 417
Journal Article
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, ISSN 2167-8421, 01/2019, Volume 20, Issue 1-2, pp. 1 - 11
Objectives: Cognitive and behavioral change in Amyotrophic Lateral Sclerosis (ALS) is well-accepted. Several screening tools have been developed to detect such... 
Amyotrophic Lateral Sclerosis | validity | cognition | behavior | screening tests | CONSENSUS | SYMPTOMS | CLINICAL NEUROLOGY | IMPAIRMENT | ECAS | INVENTORY | VERSION | DYSFUNCTION | DIAGNOSTIC-CRITERIA | ALS-FTD-Q | TOOL
Journal Article
Molecular Cell, ISSN 1097-2765, 11/2017, Volume 68, Issue 3, pp. 479 - 490.e5
Transcription of expanded microsatellite repeats is associated with multiple human diseases, including myotonic dystrophy, Fuchs endothelial corneal dystrophy,... 
myotonic dystrophy | CRISPR | RNA toxicity | transcription | microsatellite repeat disease | Cas9 | amyotrophic lateral sclerosis | RNA polymerase II | C9ORF72/ALS/FTD | STAPHYLOCOCCUS-AUREUS CAS9 | STEM-CELLS | MUSCLEBLIND PROTEINS | SMALL-MOLECULE | BIOCHEMISTRY & MOLECULAR BIOLOGY | MOUSE MODEL | MUSCULAR-DYSTROPHY | GENE-EXPRESSION | MYOTONIC-DYSTROPHY | RNA FOCI | HUNTINGTON-DISEASE | CELL BIOLOGY | C9orf72 Protein - genetics | Dependovirus - genetics | Myotonic Dystrophy - metabolism | ran GTP-Binding Protein - genetics | Alternative Splicing | RNA, Guide - genetics | CD24 Antigen - metabolism | Humans | Male | Chloride Channels - genetics | Endonucleases - metabolism | Myoblasts - metabolism | Myotonic Dystrophy - pathology | RNA, Guide - biosynthesis | HEK293 Cells | CD24 Antigen - genetics | Myotonic Dystrophy - genetics | Female | Transcription, Genetic | C9orf72 Protein - metabolism | Microsatellite Repeats | Disease Models, Animal | Transduction, Genetic | Down-Regulation | Mice, Transgenic | ran GTP-Binding Protein - metabolism | Myotonic Dystrophy - therapy | Myoblasts - pathology | Chloride Channels - metabolism | Animals | CRISPR-Associated Proteins - metabolism | CRISPR-Cas Systems | Enzyme Activation | HeLa Cells | Genetic Vectors | Genetic Therapy - methods | Enzymes | Genetic transcription | RNA | Health aspects | Analysis | Microbiology
Journal Article