Results 71 to 80 of about 24,976 (233)

Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes. [PDF]

, 2019
Frontotemporal dementia (FTD), a neurodegenerative disease broadly characterized by socioemotional impairments, includes three clinical subtypes: behavioral variant FTD (bvFTD), semantic variant primary progressive aphasia (svPPA) and non-fluent variant ...
Brown, Casey L, Casey, James J, Chen, Kuan-Hua, Hua, Alice Y, Levenson, Robert W, Lwi, Sandy J, Miller, Bruce L, Rosen, Howard J   +7 more
core  

Rapidly progressive atypical parkinsonism associated with frontotemporal lobar degeneration and motor neuron disease [PDF]

, 2010
Objective To report the rare but distinct clinical and neuropathological phenotype of non-familial, rapidly progressive parkinsonism and dementia associated with frontotemporal lobar degeneration with motor neuron disease (FTLD-MND).
de Courten-Myers, Gabrielle M., Espay, Alberto J., Ghetti, Bernardino, Houghton, David J., Litvan, Irene, Murrell, Jill R., Spina, Salvatore   +6 more
core   +1 more source

Epilepsy in dentatorubral–pallidoluysian atrophy: A systematic review and meta‐analysis

Epilepsia, EarlyView.
Summary of key clinical and electrophysiological characteristics of DRPLA‐related epilepsy from a systematic review and meta‐analysis of 1,191 patients. DRPLA patients with epilepsy showed earlier disease onset, longer CAG repeat expansion, and a tendency toward paternal inheritance. EEG findings frequently included photoparoxysmal responses.
Toru Horinouchi, Haruka Ishibashi, Yukako Nakagami, Yoko Kobayashi Takahashi, Takato Akiba, Masaharu Miyauchi, Naohiro Yamamoto, Ryoichi Inoue, Satoshi Kodama, Takafumi Kubota, Naoto Kuroda   +10 more
wiley   +1 more source

Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity

Nature Communications, 2018
TDP-43 is associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitinated inclusions (FTD-TDP). Here, the authors identify the transcriptional elongation factor Ell as a strong modifier of TDP-43-mediated ...
Chia-Yu Chung, Amit Berson, Jason R. Kennerdell, Ashley Sartoris, Travis Unger, Sílvia Porta, Hyung-Jun Kim, Edwin R. Smith, Ali Shilatifard, Vivianna Van Deerlin, Virginia M.-Y. Lee, Alice Chen-Plotkin, Nancy M. Bonini   +12 more
doaj   +1 more source

Association of cortical and subcortical microstructure with disease severity: impact on cognitive decline and language impairments in frontotemporal lobar degeneration

Alzheimer’s Research & Therapy, 2023
Background Cortical and subcortical microstructural modifications are critical to understanding the pathogenic changes in frontotemporal lobar degeneration (FTLD) subtypes. In this study, we investigated cortical and subcortical microstructure underlying
Wencai Ding, Peng Ren, Liye Yi, Yao Si, Fan Yang, Zhipeng Li, Hongbo Bao, Shi Yan, Xinyu Zhang, Siyang Li, Xia Liang, Lifen Yao, for the Frontotemporal Lobar Degeneration Neuroimaging Initiative   +12 more
doaj   +1 more source

E3 ligase Praja1 mediates ubiquitination and degradation of microtubule‐associated protein tau

The FEBS Journal, EarlyView.
E3 ligase Praja1, but not its paralogue Praja2, recognizes and ubiquitinates tau protein for proteasomal degradation. This newly identified function of Praja1‐mediated tau degradation suggests its role in protein quality control, which may provide insights into the pathogenesis of tauopathies.
Shiho Aoki, Wataru Onodera, Akihiko Takashima, Kotaro Kawasaki, Kazuki Imadegawa, Hikaru Kurahashi, Mizuho Oishi, Toru Asahi, Yoshiyuki Soeda   +8 more
wiley   +1 more source

Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration

Acta Neuropathologica Communications, 2017
An intronic GGGGCC expansion in C9orf72 is the most common known cause of both frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The repeat expansion leads to the generation of sense and antisense repeat RNA aggregates and
Sarah Mizielinska, Charlotte E. Ridler, Rubika Balendra, Annora Thoeng, Nathan S. Woodling, Friedrich A. Grässer, Vincent Plagnol, Tammaryn Lashley, Linda Partridge, Adrian M. Isaacs   +9 more
doaj   +1 more source

FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations [PDF]

, 2017
Accumulation of the DNA/RNA binding protein fused in sarcoma as cytoplasmic inclusions in neurons and glial cells is the pathological hallmark of all patients with amyotrophic lateral sclerosis with mutations in FUS as well as in several subtypes of ...
Ang, Lee-Cyn, Ansorge, Olaf, Bentmann, Eva, Bilbao, Juan, DeJesus-Hernandez, Mariely, Dormann, Dorothee, Haass, Christian, Jawaid, Ali, Kretzschmar, Hans A., Kusaka, Hirofumi, Mackenzie, Ian R. A., Munoz, David G., Neumann, Manuela, Rademakers, Rosa, Roeber, Sigrun, Yokota, Osamu   +15 more
core  

Novel diagnostic cerebrospinal fluid biomarkers for pathologic subtypes of frontotemporal dementia identified by proteomics [PDF]

, 2016
Introduction: Reliable cerebrospinal fluid (CSF) biomarkers enabling identification of frontotemporal dementia (FTD) and its pathologic subtypes are lacking.
Beccari, T. (Tommaso), Dopper, E.G.P. (Elise), Durieux-Lu, S. (Sisi), Elias, N. (Naura), Jimenez, C.R. (Connie), Koel-Simmelink, M.J.A. (Marleen), Malekzadeh, A. (Arjan), Meeter, L.H.H. (Lieke), Pham, T.V. (Thang V.), Piersma, S.R. (Sander R.), Pijnenburg, Y.A.L. (Yolande), Swieten, J.C. (John) van, Teunissen, C.E. (Charlotte)   +12 more
core   +1 more source

Co‐localization of tau and TDP‐43 after extracellular vesicle delivery to cells

The FEBS Journal, EarlyView.
Extracellular vesicles (EVs) derived from donor cells transfected with EGFP–2N4R‐tau or mCherry‐wtTDP‐43 were taken up by recipient cells, leading to cytosolic co‐localization of tau and TDP‐43. Molecular modeling revealed that tau and TDP‐43 directly interact through hydrogen bonding, suggesting a mechanistic link underlying their co‐pathology ...
Farhang Aliakbari, Kathryn Volkening, Zahra Nayeri, Aysegul Yucel Polat, Neil Donison, Jacqueline Palik, Michael J. Strong   +6 more
wiley   +1 more source