Results 201 to 210 of about 49,177 (260)

Altered mitochondria-associated ER membrane (MAM) function shifts mitochondrial metabolism in amyotrophic lateral sclerosis (ALS). [PDF]

Nat Commun
Larrea D, Tamucci KA, Kabra K, Velasco KR, Yun TD, Pera M, Montesinos J, Agrawal RR, Paradas C, Smerdon JW, Lowry ER, Stepanova A, Yoval-Sanchez B, Galkin A, Wichterle H, Area-Gomez E.   +15 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

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

Patterns of altered in vivo brain metabolism in patients with amyotrophic lateral sclerosis (ALS) and asymptomatic C9orf72 mutation carriers: a cross-sectional ¹H and ³¹P magnetic resonance spectroscopic 7T imaging study. [PDF]

EBioMedicine
Westeneng HJ, Nitert AD, van Veenhuijzen K, Wismans C, Donatelli G, Tan HHG, van Hoek W, van Es MA, Klomp DWJ, Bhogal AA, Veldink JH, Wijnen JP, van den Berg LH.   +12 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

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

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