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SCC467 MEF SQSTM1G427R Cell Line

The MEF SQSTM1G427R cell line contains a FLAG-tagged SQSTM1 protein in addition to the G427R mutation which can be used to study the underlying mechanisms of selective autophagy, ALS-FTLD disease path

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≥1X10⁶ cells/vial   
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      Overview

      Description
      Catalogue NumberSCC467
      DescriptionMEF SQSTM1G427R Cell Line
      Alternate Names
      • MEF SQSTM1 G427R
      • MEF SQSTM1
      • MEF SQSTM1G427R
      Background InformationAmyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD) are complex major neurodegenerative diseases that are not fully understood. ALS is characterized by loss of motor neurons in the brain and spinal cord while FTLD is typically characterized by degeneration of neurons in the frontal and anterior temporal lobes. Further understanding of both disorders has led them to be categorized under part of the same disease spectrum. They also share common features such as protein and protein-RNA aggregation in motor neurons. This ultimately implicates a problem in key autophagy pathways.

      Recent data suggests that TBK1 and SQSTM1 mutations linked with ALS and FTLD disrupt selective autophagy, resulting in neurotoxicity.1 SQSTM1 is an autophagy receptor protein that mediates the selective autophagy of damaged cell components or proteins that frequently aggregate. The commonality of SQSTM1-associated neuronal inclusions in ALS-FTLD patients appears to indicate its importance as a cellular stress response in which SQSTM1 protein is overproduced to try and clear protein aggregates. It also functions in other signaling pathways such as oxidative stress which has emerged as a contributing factor in ALS.1 SQSTM1 helps regulate oxidative stress through the KEAP1-NFE2L2 pathway in which SQSTM1 binds with KEAP1 resulting in a downstream antioxidant response. Interestingly, a G427R mutation appears to significantly disrupt this pathway, resulting in a highly impaired NFE2L2 response.

      The MEF (Mouse Embryonic Fibroblast) SQSTM1G427R cell line contains a FLAG-tagged SQSTM1 protein in addition to the G427R mutation which affects the Ubiquitin Binding Associated (UBA) domain of the SQSMT1 protein. This cell line can be utilized to continue studying the underlying mechanisms of selective autophagy, ALS-FTLD disease pathogenesis, and oxidative stress mechanisms. Furthermore, understanding of SQSTM1 and ALS-FTLD related mutations remains highly important as neurodegenerative diseases continue to increase in prevalence.

      Source
      Cell line was derived from immortalized p62/SQSTM1-deficient mouse embryonic fibroblasts (MEFs) which were established by infecting MEFs with a recombinant retrovirus carrying a temperature-sensitive simian virus 40 large T antigen. Flag-SQSTM1-WT was also cloned into the HindIII and Xhol sites of the LPC retroviral vector before undergoing site-directed mutagenesis to obtain the SQSTM1G427R genotype.

      References
      Deng Z, Lim J, Wang Q, Purtell K, Wu S, Palomo GM, Tan H, Manfredi G, Zhao Y, Peng J, et al. 2019 Jul 30. ALS-FTLD-linked mutations of SQSTM1/p62 disrupt selective autophagy and NFE2L2/NRF2 anti-oxidative stress pathway. Autophagy.:1–15.
      Product Information
      Components
      • ≥1x106 viable MEF SQSTM1G427R cells (Catalog No. SCC467). Store in liquid nitrogen.
      Quality LevelMQ100
      Applications
      ApplicationThe MEF SQSTM1G427R cell line contains a FLAG-tagged SQSTM1 protein in addition to the G427R mutation which can be used to study the underlying mechanisms of selective autophagy, ALS-FTLD disease pathogenesis, and oxidative stress mechanisms.
      Key Applications
      • Cell Culture
      • Neuroscience
      Application NotesThis product is intended for sale and sold solely to academic institutions for internal academic research use per the terms of the “Academic Use Agreement” as detailed in the product documentation. For information regarding any other use, please contact licensing@milliporesigma.com.
      Biological Information
      HostMouse
      Cell Line Type
      • Fibroblast Cells
      Product Usage Statements
      Quality Assurance• Each vial contains ≥ 1X106 viable cells.
      • Cells are tested negative for infectious diseases by a Mouse Essential CLEAR Panel by Charles River Animal Diagnostic Services.
      • Cells are verified to be of mouse origin and negative for interspecies contamination from human, rat, Chinese hamster, Golden Syrian hamster, and nonhuman primate (NHP) as assessed by a Contamination Clear panel by Charles River Animal Diagnostic Services
      • Cells are negative for mycoplasma contamination.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      • This product contains genetically modified organisms (GMO). Within the EU GMOs are regulated by Directives 2001/18/EC and 2009/41/EC of the European Parliament and of the Council and their national implementation in the member States respectively. This legislation obliges Merck to request certain information about you and the establishment where the GMOs are being handled. Click here for Enduser Declaration (EUD) Form.
      Storage and Shipping Information
      Storage ConditionsMEF SQSTM1G427R cells should be stored in liquid nitrogen. The cells can be cultured for at least 10 passages without significantly affecting cell marker expression and function.
      Packaging Information
      Material Size≥1X10⁶ cells/vial
      Global Trade Item Number
      Catalogue Number GTIN
      SCC467 04065271938403