Innoprot offers several Amyotrophic Lateral Sclerosis in vitro disease models that address the disease through different associated cellular pathways; including TDP-43 and FUS/TLS stress granules formation and glutamate damage induction.
About 10% of ALS cases are inherited; and a large subset of them are caused by mutations in the gene encoding the copper-zinc superoxide dismutase (SOD1). The detection of SOD1-positive inclusions in familial ALS patients suggests the role of SOD1 aggregation underlying the pathology of familial ALS. Although SOD1 mutant proteins are different in structure,…
Amyotrophic lateral sclerosis (ALS) is one of the most common degenerative disease of the motor neuron system. The TDP-43 pathology seems to be a dominant type of pathology across sporadic ALS types. Our TDP-43 Stress Granules Assay allows the quantification of pathological TDP43 globs into the nucleus and cytosol. This model also permits to monitor…
FUS/TLS Stress Granules Assay from Innoprot requires a stable cell line expressing fluorescent FUS/TLS. During the assay, we incubate cells with test compounds and Sodium Arsenite to induce the FUS/TLS aggregation. Finally, we quantify fluorescent FUS/TLS nuclear globs using the BD Pathway HCS Reader and Attovision Compartimentalization Software. Amyotrophic lateral sclerosis (ALS) is one of…
A brief exposure to glutamate in vitro causes neuronal death, which makes glutamate a useful tool to evaluate neuroprotective activities. Innoprot excitotoxicity in vitro assay offers the advantage of a rapid analysis of the neuroprotective potential of a molecule. In our assay, we incubate rat primary neurons with test compounds 24 hours before L-glutamate insult.…