Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by loss of both; upper and lower motoneurons in the brain and the spinal cord. ALS and other neurodegenerative disorders, such as Alzheimer\u2019s and Parkinson\u2019s disease, are characterized by defects in protein processing resulting in protein misfolding, mislocalization and inclusion formation in motor neurons. Classical neuropathological hallmarks of ALS include ubiquitinated inclusions containing the disordered TDP-43 and FUS proteins; although pathology can be heterogeneous with the appearance of other protein aggregates. Mutations in more than forty genes have been reported to associate with ALS.<\/p>\n
Mutations in the gene encoding superoxide dismutase 1 (SOD1) were the first discovered to cause ALS, and elicit approximately 20% of all familiar ALS cases in the world and up to 50% in China. The alanine to valine mutation at codon 4 (A4V) of SOD1 causes a rapidly progressive dominant form of ALS with exclusively lower motor neuron disease. It is also one of the most common mutations worldwide responsible for 50% of SOD1 mutations associated with familial ALS. Usually, the genes associated with ALS are related with misfolded and aggregated proteins. In the case of SOD1-A4V mutant, it has an increased tendency to aggregate and form aberrant depositions. Misfolded and aggregated SOD1-A4V elicits many toxic properties and has been linked with cell death. SOD1 A4V Aggregation Assay Cell Line is a useful tool to assay compounds to modulate SOD1 tendency to form aggregates.<\/p>\n
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Innoprot has developed a novel fluorescence cell-based assay cell line for High Content Screening that allows the quantification of SOD1 aggregation on living cells. This model consists in a HEK293 cell line stably expressing red fluorescent A4V mutant of SOD1<\/strong>. This cell line has been designed to assay compounds or analyze their capability to modulate superoxide dismutase 1 tendency to form aggregates inside the cell. The addition of proteasome inhibitor ALLN, produces the aggregation of the protein in HEK293 cells. Co-incubation with test-compounds, allows the identification of potential inhibitors of SOD1 aggregation.<\/p>\n Innoprot offers this cell line as a \u201cstable cell line\u201d in cryopreserved vials. Each vial of\u00a0HEK293 cell line stably expressing red fluorescent tagged (TurboFP602) SOD1-A4V protein contains 3 million cells and Innoprot provides 2 vials of cells with each order. These cells proliferate in vitro and they are guaranteed to further expand for more than 30 passages following the instructions provided in the technical sheet.<\/p>\n Size\/Quantity<\/strong>: 2 vials containing 3×106<\/sup> cells \/ vial<\/p>\n","protected":false},"featured_media":8294,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false},"product_brand":[],"product_cat":[115],"product_tag":[],"class_list":["post-8293","product","type-product","status-publish","has-post-thumbnail","product_cat-stable-cell-lines-for-drug-screening-in-cns","first","instock","taxable","shipping-taxable","purchasable","product-type-simple"],"acf":[],"yoast_head":"\n