Results 61 to 70 of about 5,310,608 (313)

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3 [PDF]

, 2008
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion in ataxin-3 (SCA3, MJD1) protein.
X. Chen, Bezprozvanny, I, Nelson, O, Hammer, R, Tu, H, Nukina, N, O. Nelson, N. Nukina, Chen, X, Tang, TS, T.-S. Tang, Pook, M, H. Tu, R. Hammer, I. Bezprozvanny, M. Pook   +15 more
core   +1 more source

Retained capacity for perceptual learning of degraded speech in primary progressive aphasia and Alzheimer’s disease

Alzheimer’s Research & Therapy, 2018
Background Processing of degraded speech is a promising model for understanding communication under challenging listening conditions, core auditory deficits and residual capacity for perceptual learning and cerebral plasticity in major dementias. Methods
Chris J. D. Hardy, Charles R. Marshall, Rebecca L. Bond, Lucy L. Russell, Katrina Dick, Cono Ariti, David L. Thomas, Sonya J. Ross, Jennifer L. Agustus, Sebastian J. Crutch, Jonathan D. Rohrer, Doris-Eva Bamiou, Jason D. Warren   +12 more
doaj   +1 more source

Effect of terahertz irradiation on DNA damage repair in living cells

FEBS Open Bio, EarlyView.
We investigated the effect of terahertz (THz) wave irradiation on DNA double‐strand break (DSB) repair in living cells. We found that THz irradiation enhanced DSB repair at specific frequencies, whereas heat treatment inhibited it, indicating that this effect is nonthermal and frequency‐specific.
Yuya Ueno, Shota Yamazaki, Hiromichi Hoshina, Masahiko Harata   +3 more
wiley   +1 more source

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability [PDF]

, 2015
This work has been supported by: Motor Neurone Disease Association (G.B.M., S.C. and C.E.S.); Euan MacDonald Centre (G.B.M. and S.C.); European Research Council (L.V.); Cambridge Hospitals National Institute for Health Research Biomedical Research Center
Foster, Joshua D., Shaw, Christopher E., Cleary, Elaine M., Cleary, Elaine M, Foster, Joshua D, Miles, Gareth B, Devlin, Anna-Claire, Geti, Imbisaat, Cleary, Elaine Marie, Miles, Gareth B., Borooah, Shyamanga, Devlin, Anna Claire, Shaw, Christopher E, Vallier, Ludovic, Burr, Karen, Chandran, Siddharthan   +15 more
core   +1 more source

MARK4 enhances stress granule formation under oxidative stress and increases tau accumulation

FEBS Open Bio, EarlyView.
MARK4 (red dots) localizes to stress granules (orange dots) and promotes their formation under oxidative stress by modulating TIA1 (blue dots). MARK4 and TIA1 synergistically increase tau (purple) accumulation, and the reduction of the TIA1 ortholog suppresses neurodegeneration in a fly model.
Sho Nakajima, Kotone Watanabe, Grigorii Sultanakhmetov, Aoi Fukuchi, Keiya Ito, Sawako Shimizu, Taro Saito, Akiko Asada, Kanae Ando   +8 more
wiley   +1 more source

CSPα in neurodegenerative diseases

Frontiers in Aging Neuroscience, 2022
Adult-onset neuronal ceroid lipofuscinosis (ANCL) is a rare neurodegenerative disease characterized by epilepsy, cognitive degeneration, and motor disorders caused by mutations in the DNAJC5 gene. In addition to being associated with ANCL disease, the cysteine string proteins α (CSPα) encoded by the DNAJC5 gene have been implicated in several ...
Liqin Huang, Zhaohui Zhang
openaire   +3 more sources

Pharmacotherapy of Neurodegenerative Diseases

, 2022
Amyotrophic Lateral Sclerosis (ALS) is a fatal idiopathic neurodegenerative disease that is characterized by degeneration of both upper and lower motor neurons. ALS currently only has 2 drugs approved as disease modyfying, whereas the rest of the therapy
Adem, Muna Abdu
core  

Specific biomarkers for C9orf72 FTD/ALS could expedite the journey towards effective therapies

EMBO Molecular Medicine, 2017
A hexanucleotide repeat expansion in the C9orf72 gene is a common genetic cause of ALS and FTD. The repeats are translated into five different dipeptide repeat proteins (DPRs).
Rubika Balendra, Thomas G Moens, Adrian M Isaacs   +2 more
doaj   +1 more source

Prominent amyloid plaque pathology and cerebral amyloid angiopathy in APP V717I (London) carrier – phenotypic variability in autosomal dominant Alzheimer’s disease

Acta Neuropathologica Communications, 2020
The discovery of mutations associated with familial forms of Alzheimer’s disease (AD), has brought imperative insights into basic mechanisms of disease pathogenesis and progression and has allowed researchers to create animal models that assist in the ...
Grace M. Lloyd, Jorge A. Trejo-Lopez, Yuxing Xia, Karen N. McFarland, Sarah J. Lincoln, Nilüfer Ertekin-Taner, Benoit I. Giasson, Anthony T. Yachnis, Stefan Prokop   +8 more
doaj   +1 more source

TRPML1 agonist ML‐SA5 attenuates pulmonary fibroblast activation by suppressing mTOR and restoring autophagic flux

FEBS Open Bio, EarlyView.
TGF‐β1 stimulation downregulates lysosomal channel TRPML1 in pulmonary fibroblasts. The TRPML1 agonist ML‐SA5 suppressed fibroblast‐to‐myofibroblast activation and collagen production. Mechanistically, ML‐SA5 inhibited mTOR phosphorylation, restored autophagic flux, and its effects were enhanced by rapamycin (mTOR inhibitor) and reversed by MHY1485 ...
Jiatong Yao, Xiaoting Kang, Lulu Meng, Shaoxuan Zhang, Qiujie Li, Jinyu Zhou, Mengyue Xue, Chu Li, Hua Zhu, Pengzhou Hang, Jing Zhao   +10 more
wiley   +1 more source