Results 61 to 70 of about 21,388 (227)

Advances in the Understanding of Frontotemporal Dementia

Cells, 2023
Frontotemporal dementia (FTD) encompasses a group of clinically, genetically and pathologically heterogeneous neurodegenerative disorders that mainly affect people under the age of 64 years [...]
Rina Bandopadhyay, Ariana Gatt, Tammaryn Lashley   +2 more
doaj   +1 more source

Sex‐Stratified Association of Regional Dopamine Transporter Binding With Disease Progression in Amyotrophic Lateral Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To clarify the clinical relevance of dopamine transporter single‐photon emission computed tomography (DAT‐SPECT) abnormalities in amyotrophic lateral sclerosis (ALS), with a prespecified focus on sex‐stratified associations with disease progression and short‐term prognosis.
Tomoya Kawazoe, Keizo Sugaya, Kentaro Hayashi, Keiko Hino, Yasuhiro Nakata, Kota Bokuda, Toshio Shimizu, Kazushi Takahashi   +7 more
wiley   +1 more source

A 57‐Year‐Old Male With Behavioral Variant Frontotemporal Dementia and MATR3 and NOS3 Mutations

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT This report presents a case of behavioral variant frontotemporal dementia caused by mutations in the MATR3 and NOS3 genes, aiming to analyze its clinical manifestations and genetic characteristics. For a case presenting with personality changes and gait abnormalities as the initial symptoms, this study conducted a comprehensive analysis of its
Feifei Lin, Saie Huang
wiley   +1 more source

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

, 2014
An expanded GGGGCC repeat in C9orf72 is the most common genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis. A fundamental question is whether toxicity is driven by the repeat RNA itself and/or by dipeptide repeat proteins ...
Ridler, CE, Pickering-Brown, Stuart, Norona, FE, Woollacott, I., Fratta, P., Norona, Frances E, Woollacott, IOC, Ridler, Charlotte E, Devoy, A, Pietrzyk, J, Fisher, EMC, Devoy, Anny, Dols, J, Dols, Jacqueline, Moens, T., Cleverley, K., Fratta, Pietro, Partridge, Linda, Nicoll, Andrew J, Fisher, E., Ridler, C., Fratta, P, Grönke, S., Pickering-Brown, S., Isaacs, Adrian M, Cabecinha, Melissa, Mizielinska, S, Moens, Thomas, Clayton, E., Partridge, L., Pietrzyk, J., Clayton, Emma L, Mizielinska, Sarah, Niccoli, T., Devoy, Anny; id_orcid, Mizielinska, Sarah; id_orcid, Isaacs, A., Woollacott, Ione O C, Cleverley, Karen, Nicoll, AJ, Hendrich, O., Moens, T, Dols, J., Grönke, Sebastian, Cabecinha, M., Cabecinha, M, Fisher, Elizabeth M C, Mizielinska, S., Clayton, EL, Nicoll, A., Hendrich, Oliver, Grönke, S, Niccoli, Teresa, Pietrzyk, Julian, Devoy, A., Norona, F., Isaacs, AM, Pickering-Brown, S, Cleverley, K, Hendrich, O, Niccoli, T, Partridge, L   +61 more
core   +1 more source

Vestibular Impairment in Frontotemporal Dementia Syndrome

Dementia and Geriatric Cognitive Disorders Extra, 2016
Background: No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. Objective: The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical ...
Kiyotaka Nakamagoe, Kotarou Kadono, Tadachika Koganezawa, Mao Takiguchi, Makoto Terada, Fumiko Yamamoto, Tetsuya Moriyama, Kumi Yanagiha, Seitaro Nohara, Naoki Tozaka, Zenshi Miyake, Satoshi Aizawa, Kentaro Furusho, Akira Tamaoka   +13 more
doaj   +1 more source

Orthogonal Ionic Liquid‐Based Extraction Strategy Enables Amyloid‐Specific Profiling of Aggregate Proteome

Advanced Science, EarlyView.
An orthogonal ionic‐liquid extraction (Orth‐iEA) enables selective isolation of amyloid fibrils. TMGBF4 disrupts hydrogen‐bonded β‐sheet networks to solubilize amyloid aggregates, whereas C12ImCl interacts with hydrophobic regions of non‐amyloid proteins.
Shiying Zheng, Ye Liu, Bowen Zhong, Huiying Chu, Zhou Gong, Baofeng Zhao, Mengchun Cheng, Zhen Liang, Yukui Zhang, Qun Zhao, Lihua Zhang   +10 more
wiley   +1 more source

Recent advances in frontotemporal dementia

, 2016
There have been major advances in the characterisation of frontotemporal dementia (FTD) over the past two decades culminating in the development of internationally accepted diagnostic criteria for each of the clinical ...
John R Hodges, Emma Devenney
core   +1 more source

TDP‐43 Aggregation: The Healthy‐Toxic Balance of the Prion‐Like Domain

Advanced Science, EarlyView.
TDP‐43 function relies on a delicate balance between reversible phase‐separated states and irreversible aggregation. Under physiological conditions, TDP‐43 forms dynamic droplets and oligomers that support normal cellular functions. In pathological contexts, this balance shifts toward aberrant aggregation, leading to toxic species.
Luca Zangrando, Emanuele Buratti, Francesca Paron   +2 more
wiley   +1 more source

Progranulin Leu271LeufsX10 is one of the most common FTLD and CBS associated mutations worldwide

Neurobiology of Disease, 2009
Mutations in the progranulin gene (PGRN) are a major cause of frontotemporal lobar degeneration (FTLD). Herein we estimated the contribution of the PGRN Leu271LeufsX10 mutation to FTLD and related disorders in the Brescia cohort.
Luisa Benussi, Roberta Ghidoni, Eleonora Pegoiani, Davide V. Moretti, Orazio Zanetti, Giuliano Binetti   +5 more
doaj   +1 more source

Frontotemporal Dementia Rating Scale (FTD-FRS): Linguistic and Cultural Adaptation in Russia

Анналы клинической и экспериментальной неврологии, 2020
Introduction. Frontotemporal dementia (FTD) is a neurodegenerative disease and is the second most frequent cause of early-onset dementia (before 65 years).
Yuliya A. Shpilyukova, Djamilya G. Yusupova, Diliara R. Akmadullina, Natalia V. Polekhina, Alexander V. Naminov, Mikhail E. Sinelnikov, Aleksander B. Zaytsev, Aleksey A. Zimin, Anastasia V. Belopasova, Ekaterina Yu. Fedotova, Nataliya A. Suponeva, Sergey N. Illarioshkin, Mikhail A. Piradov   +12 more
doaj   +1 more source