ALS
- The transcription factor Nurr1 is upregulated in amyotrophic lateral sclerosis patients and SOD1-G93A mice
Summary: We hypothesize that the transcription factor Nurr1 is activated in the early phase of the neurodegenerative disease amylotrophic lateral sclerosis (ALS), probably as a neuroprotective endogenous mechanism. Nurr1 might represent a promising target for ALS therapy.
- The expanding spectrum of neurological disorders of phosphoinositide metabolism
Summary: This Review highlights the intersection between phosphoinositides and the enzymes that regulate their metabolism, which together are crucial regulators of myriad cellular processes and neurological disorders.
- SOD1 activity threshold and TOR signalling modulate VAP(P58S) aggregation via reactive oxygen species-induced proteasomal degradation in a Drosophila model of amyotrophic lateral sclerosis
Summary: ALS8/VAPB(P58S) aggregates appear to be cleared by an increase in cellular reactive oxygen species, which, in turn, triggers proteasomal degradation.
- Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis
Summary: This Review compares two key types of amyotrophic lateral sclerosis (ALS) mouse models: transgenic mice and those that express genes at physiological levels. We explore the advantages of each type for studying pathomechanisms, to understand what they can teach us about ALS.
- A differential autophagy-dependent response to DNA double-strand breaks in bone marrow mesenchymal stem cells from sporadic ALS patients
Summary: A novel endogenous disease mechanism in cells from ALS patients after NCS-induced DNA damage.
- A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects
Summary: An engineered TDP-43 construct can be used to induce TDP-43 aggregation in Drosophila, providing a model that could be useful for characterization of pathogenetic mechanisms and drug screening.
- A natural human IgM that binds to gangliosides is therapeutic in murine models of amyotrophic lateral sclerosis
Summary: A single peripheral dose of a recombinant natural human IgM increases lifespan and delays neurological deficits in mouse models of human ALS.
- ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons
Summary: Mutated FUS protein is aberrantly delocalized and recruited into stress granules in iPSC-derived motoneurons, which provide a new model system for amyotrophic lateral sclerosis.