Results 201 to 210 of about 995,258 (307)

Thyroid hormones and energy metabolism in amyotrophic lateral sclerosis. [PDF]

Brain Commun
Novikova L, Lang C, Tumani H, Klose V, Kassubek J, Dreyhaupt J, Dupuis L, Wabitsch M, Ludolph A.   +8 more
europepmc   +1 more source

Proteostasis of organelles in aging and disease

The FEBS Journal, EarlyView.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

Distinct cellular phenotypes of language and executive decline in amyotrophic lateral sclerosis. [PDF]

Cell
Petrescu J, Gouveia Roque C, Jackson CA, Daly AC, Butti Z, Kang K, Casel O, Leung M, Reilly L, Eschbach J, Gebremedhin B, McDade K, Gregory JM, Bonneau R, Smith C, Phatnani H.   +15 more
europepmc   +1 more source

Bringing cellular clarity to the cortical component of ALS with a high‐density multi‐electrode array system

The FEBS Journal, EarlyView.
High density multi‐electrode (HD‐MEA) systems enable detailed investigation of healthy and diseased brains in 2D and 3D. Here, we summarise the significance of single‐cell electrophysiology, advantages of the MEA systems with high spatio‐temporal resolution and a large number of recording sites, enabling complex network analysis.
Zehra Yagmur Erol, Christopher Quintanilla, P. Hande Ozdinler   +2 more
wiley   +1 more source

Cortical excitability stratifies neurochemical profiles in amyotrophic lateral sclerosis. [PDF]

Brain Commun
Tu S, Mahoney CJ, Huynh W, Vucic S, Kiernan MC.   +4 more
europepmc   +1 more source

Biogenesis of TNF‐α‐insights into proteostasis and inflammation

The FEBS Journal, EarlyView.
TNF‐α biogenesis, trafficking, and signalling are tightly and reciprocally coupled to cellular proteostasis systems, including ER chaperones and endoplasmic reticulum‐associated degradation. This bidirectional crosstalk determines whether TNF‐α responses are adaptive or proteotoxic.
Bailasan Haidar, Céline Philippe, Eric Chevet, Jiří Zahradník   +3 more
wiley   +1 more source

Central nervous system concentrations of nano- and microplastics and risk of amyotrophic lateral sclerosis. [PDF]

Brain Commun
Vinceti M, Ferrante M, Filippini T, Wise LA, Urbano T, Bedin R, Gianferrari G, Rapisarda P, Pulvirenti E, Oliveri Conti G, Mandrioli J.   +10 more
europepmc   +1 more source

Iloperidone treatment mitigates the Juvenile Huntington's Disease phenotype possibly via Sigma‐1 Receptor Modulation

The FEBS Journal, EarlyView.
We investigated the potential of iloperidone as an activator of Sigma‐1 receptor (S1R) neuroprotective function in juvenile Huntington's disease (jHD). We tested iloperidone on cortical neurons differentiated from patient‐derived iPSCs, demonstrating that it acts as a S1R agonist, decreasing apoptosis, huntingtin aggregation, and oxidative stress ...
Ersilia Fornetti, Gaia Galluzzi, Mario Frezzini, Cécile Exertier, Vittorio Brufani, Gianni Colotti, Giancarlo Ruocco, Jessica Rosati, Angelo Luigi Vescovi, Daniele Narzi, Ilaria Genovese, Andrea Ilari   +11 more
wiley   +1 more source

Unraveling in vitro phase separation and aggregation properties of the structured region of FMRP and the impact of Fragile X syndrome‐linked mutations

The FEBS Journal, EarlyView.
Fragile X messenger ribonucleoprotein 1 (FMRP) is a multidomain RNA‐binding protein associated with Fragile X Syndrome (FXS). We found that its N‐terminal structured region has an intrinsic propensity to undergo liquid–liquid phase separation and fibril formation. FXS‐associated mutations perturb protein stability and aggregation propensity, suggesting
Flavia Catalano, Alessandra Bigi, Francesca Troilo, Federica Gabriele, Gianluca Pistoia, Italia A. Asteriti, Francesco Angelucci, Giorgio Giardina, Fabrizio Chiti, Carlo Travaglini‐Allocatelli, Adele Di Matteo   +10 more
wiley   +1 more source

Myofibrillar Myopathy with Pyramidal Signs Mimicking Amyotrophic Lateral Sclerosis. [PDF]

Ann Indian Acad Neurol
Mirg S, Elavarasi A, Das A, Vibha D, Singh RK, Parihar J, Sharma MC, Tripathi M.   +7 more
europepmc   +1 more source