Results 241 to 250 of about 95,709 (300)

Use of Muscle Ultrasonography in Morphofunctional Assessment of Amyotrophic Lateral Sclerosis (ALS). [PDF]

Nutrients
López-Gómez JJ, Izaola-Jauregui O, Almansa-Ruiz L, Jiménez-Sahagún R, Primo-Martín D, Pedraza-Hueso MI, Ramos-Bachiller B, González-Gutiérrez J, De Luis-Román D.   +8 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

Clinical spreading of muscle weakness in amyotrophic lateral sclerosis (ALS): a study in 910 patients. [PDF]

J Neurol
Ludolph AC, Dietrich J, Dreyhaupt J, Kassubek J, Del Tredici K, Rosenbohm A.   +5 more
europepmc   +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

Proteolytic remodelling of the extracellular matrix by pericytes

The FEBS Journal, EarlyView.
Pericytes are specialised perivascular cells intimately connected with endothelial cells and essential for the maintenance of vascular beds. They contribute to the formation and remodelling of the extracellular matrix by actively secreting proteases and protease inhibitors.
Tina Burkhard, Ella Milne, Kate Qian, Paola Campagnolo, Salvatore Santamaria   +4 more
wiley   +1 more source

Enhanced secretion of the amyotrophic lateral sclerosis ALS-associated misfolded TDP-43 mediated by the ER-ubiquitin specific peptidase USP19. [PDF]

Cell Mol Life Sci
Picard F, Nonaka T, Belotti E, Osseni A, Errazuriz-Cerda E, Jost-Mousseau C, Bernard E, Conjard-Duplany A, Bohl D, Hasegawa M, Raoul C, Galli T, Schaeffer L, Leblanc P.   +13 more
europepmc   +1 more source

High-content analysis of proteostasis capacity in cellular models of amyotrophic lateral sclerosis (ALS). [PDF]

Sci Rep
Lambert-Smith IA, Shephard VK, McAlary L, Yerbury JJ, Saunders DN.   +4 more
europepmc   +1 more source

Diffusion tensor imaging biomarkers and clinical assessments in amyotrophic lateral sclerosis (ALS) patients: an exploratory study. [PDF]

Ann Med Surg (Lond)
Pezeshgi S, Ghaderi S, Mohammadi S, Karimi N, Ziaadini B, Mohammadi M, Fatehi F.   +6 more
europepmc   +1 more source

An Effective DNA Methylation Biomarker Screening Mechanism for Amyotrophic Lateral Sclerosis (ALS) Based on Comorbidities and Gene Function Analysis. [PDF]

Bioengineering (Basel)
Yang CH, Huang JL, Tsai LK, Taniar D, Pai TW.   +4 more
europepmc   +1 more source

Insights on Natural Products Against Amyotrophic Lateral Sclerosis (ALS). [PDF]

Curr Neuropharmacol
Monteiro KLC, Dos Santos Alcântara MG, de Aquino TM, da Silva-Júnior EF.   +3 more
europepmc   +1 more source