Results 301 to 310 of about 242,580 (385)

Restless legs syndrome as a comorbidity in amyotrophic lateral sclerosis: a systematic review and meta-analysis. [PDF]

BMC Neurol
Ebrahimian A, Moradi A, Athari SZ, Farajdokht F.   +3 more
europepmc   +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

Clinical Utility of Far-Field Potentials in Amyotrophic Lateral Sclerosis. [PDF]

Muscle Nerve
Calma AD, Pavey N, Silva CS, Tsuji Y, Van den Bos MAJ, Farrar MA, Menon P, Vucic S.   +7 more
europepmc   +1 more source

3′UTR variants of ALS‐linked RNAs modify subcellular and cellular phenotypes

The FEBS Journal, EarlyView.
Our study demonstrates that alternative 3′UTR variants of ALS‐linked transcripts modulate subcellular RNA localization and cytoskeletal architecture. NEFH 3′UTR‐Long promotes nuclear RNA clustering, while SOD1 3′UTR‐Long reduces filopodia formation. These results suggest that 3′UTRs, independent of coding sequences, can influence neuronal phenotypes ...
Melis Savasan‐Sogut, Danae Campos‐Melo, Michael J. Strong   +2 more
wiley   +1 more source

Cumulative incidence of motor and cognitive features in the amyotrophic lateral sclerosis-frontotemporal degeneration spectrum. [PDF]

Brain Commun
Spencer BE, Xie SX, Ohm DT, Elman L, Quinn CC, Amado DA, Baer M, Lee EB, Van Deerlin VM, Dratch L, Massimo L, Irwin DJ, McMillan CT.   +12 more
europepmc   +1 more source

The ALS‐associated E425K mutation uncouples DNAJC7 from the Hsp70 chaperone cycle

The FEBS Journal, EarlyView.
DNAJC7 is a J‐domain protein that plays a key role in protein quality control by regulating Hsp70 activity and preventing protein aggregation. We find that the ALS‐associated E425K mutation in DNAJC7 disrupts productive interaction and activation of Hsp70, thereby blocking the transfer and refolding of client proteins such as TDP‐43.
Bar Elmaleh, Ofrah Faust, Rina Rosenzweig   +2 more
wiley   +1 more source

Demographic, clinical and genetic characteristics of patients with amyotrophic lateral sclerosis from two specialised centres in Austria. [PDF]

J Neurol
Keritam O, Kleinveld VE, Klotz S, Caliskan H, Mayerhofer M, Sener M, Jäger F, Weng R, Bormann D, Pugna I, Gebert J, Fedak I, Renner A, Antoniewicz L, Rath J, Zulehner G, Krenn M, Zimprich F, Löscher WN, Cetin H.   +19 more
europepmc   +1 more source

Redox environment modulates aggregation of ataxin‐3 in vitro — Implications for drug screening of cysteine‐rich proteins

The FEBS Journal, EarlyView.
Redox environment modulates in vitro aggregation of Ataxin‐3, the protein implicated in spinocerebellar ataxia type 3. Reducing conditions stabilize native monomers and prevent aggregation, whereas oxidative conditions promote the formation of non‐native conformers and disulfide‐linked oligomers within the Josephin domain (JD).
Martyna Podlasiak, Martina Sollazzo, Elisa Monaca, Raffaele Sabbatella, Maria Agnese Morando, Oleg Chertkov, Laura Puglisi, Martina Mascellino, Anna Fricano, Sandra Macedo‐Ribeiro, Caterina Alfano   +10 more
wiley   +1 more source