Results 141 to 150 of about 74,541 (281)

Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis

open access: hybrid, 2014
Javier Sábado   +6 more
openalex   +1 more source

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

open access: yesThe 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   +2 more
wiley   +1 more source

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

open access: yesThe 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   +2 more
wiley   +1 more source

Effect of ELF e.m. fields on metalloprotein redox-active sites

open access: yes, 2007
The peculiarity of the distribution and geometry of metallic ions in enzymes pushed us to set the hypothesis that metallic ions in active-site act like tiny antennas able to pick up very feeble e.m. signals.
Badon, T.   +6 more
core  

Tryptophan 32 mediates SOD1 toxicity in a in vivo motor neuron model of ALS and is a promising target for small molecule therapeutics

open access: green, 2018
Michèle G. DuVal   +9 more
openalex   +2 more sources

Tui Na Acupressure Modulates Treg Immunosuppression via FoxP3/mTORC1 Signalling in ALS Mice

open access: yesImmunology, EarlyView.
The proposed mechanism by which Tui Na acupressure improves motor and cognitive dysfunction in ALS mice by activating the FoxP3/mTORC1 pathway. ABSTRACT Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease driven by neuroinflammation, where regulatory T cell (Treg) dysfunction exacerbates immune imbalance.
Jie Han   +7 more
wiley   +1 more source

A VDAC1-Derived N-Terminal Peptide Inhibits Mutant SOD1-VDAC1 Interactions and Toxicity in the SOD1 Model of ALS [PDF]

open access: gold, 2019
Anna Shteinfer‐Kuzmine   +11 more
openalex   +1 more source

Evolutionary Conserved and Divergent Responses to Copper Zinc Superoxide Dismutase Inhibition in Plants

open access: yesPlant, Cell &Environment, EarlyView.
ABSTRACT After an initial evolution in a reducing environment, life got successively challenged by reactive oxygen species (ROS), especially during the great oxidation event (GOE) that followed the development of photosynthesis. Therefore, ROS are deeply intertwined into the physiological, morphological and transcriptional responses of most present‐day
Stephanie Frohn   +12 more
wiley   +1 more source

Hypoxia-inducible factor: role in cell survival in superoxide dismutase overexpressing mice after neonatal hypoxia-ischemia. [PDF]

open access: yes, 2019
BackgroundSixty percent of infants with severe neonatal hypoxic-ischemic encephalopathy die, while most survivors have permanent disabilities. Treatment for neonatal hypoxic-ischemic encephalopathy is limited to therapeutic hypothermia, but it does not ...
Ferriero, Donna M   +2 more
core  

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