Results 231 to 240 of about 95,709 (300)

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

Resting-State EEG Oscillations in Amyotrophic Lateral Sclerosis (ALS): Toward Mechanistic Insights and Clinical Markers. [PDF]

J Clin Med
Chmiel J, Stępień-Słodkowska M.
europepmc   +1 more source

Genetic correlation between amyotrophic lateral sclerosis and schizophrenia [PDF]

, 2017
Cloninger, C. Robert, et al,, Svrakic, Dragan M   +2 more
core   +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

Efficacy of pain management strategies in adults with Amyotrophic Lateral Sclerosis (ALS): A Systematic Review. [PDF]

Neurol Sci
Rojas-López JC, Estrada-Gualdron PI, Ramírez-Guerrero S, Velásquez-Cárdenas MJ, Redondo-Escobar J, Vargas-Arenas S, Palacios-Sánchez L, Palacios-Espinosa X.   +7 more
europepmc   +1 more source

Holding but not folding: How a single charge flip uncouples the DNAJC7‐Hsp70 relay in amyotrophic lateral sclerosis

The FEBS Journal, EarlyView.
In this issue, Elmaleh et al. demonstrate that the E425K mutation in DNAJC7, associated with amyotrophic lateral sclerosis, selectively abolishes one of a co‐chaperone's dual functions. High‐resolution NMR confirmed that the mutation leaves the protein's structure intact while paralyzing its communication with the Hsp70 machinery.
Tsung‐Sheng Chiang, Jerome Boisbouvier, Lauren A. Gandy   +2 more
wiley   +1 more source

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

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

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

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