HEK293 FUS Knockout Human Cell Line

Amyotrophic lateral sclerosis (ALS, Charcot or Lou Gehrig s disease) is a progressive neurodegenerative disease resulting in loss of motor control. ALS usually leads to mortality within 5 years of symptom onset.1 A subset of ALS cases are familial and lin

Amyotrophic lateral sclerosis (ALS, also known as Charcot or Lou Gehrig′s disease) is a progressive neurodegenerative disease resulting in loss of motor control. ALS usually leads to mortality within 5 years of symptom onset.1 A subset of ALS cases are familial and linked to mutations in a handful of genes, including FUS (fused in sarcoma). FUS is a ribonucleotide binding protein that plays a role in RNA metabolism and is implicated in DNA repair pathways.2 Probing the effects of FUS point mutations is contributing to our understanding of the cellular mechanisms of ALS and the connections between genetic repair and neurodegeneration.

Source
HEK293 FUS KO cell line was derived from CRISPR-edited HEK293 cells.2 The parental HEK293 cell line was derived from cells from an aborted female fetus transfected with sheared adenovirus DNA.3

References
1. Zarei S, Carr K, Reiley L, Diaz K, Guerra O et al. Surg Neurol Int 2015; 6:171.
2. Wang H, Guo W, Mitra J, Hegde PM, Vandoorne T et al. Nat Commun 2018; 9:3683.
3. Graham FL, Smiley J, Russell, WC, Nairn R. J Gen Virol 1977; 36(1): 59-74.

The HEK293 FUS knockout cell line was derived via CRISPR/Cas9-editing of HEK293 cells. Experimental validation of this line confirmed deletion of FUS with no off-target effects on expression of related proteins.2 This FUS knockout cell line permits direct study of the effects of FUS mutations via transfection of FUS variants. The HEK293 FUS knockout cell line represents a valuable system for the investigation of familial ALS in a robust cellular model.

Cells should be stored in liquid nitrogen until use. The cells can be cultured for at least 10 passages after initial thawing without significantly affecting the cell marker expression and functionality.

Subject to local law, this product is intended to be sold for internal in vitro research use only subject to terms and conditions found here: www.sigmaaldrich.com/restrictedcelluse. This product may not be: re-engineered or copied; used to make derivatives, modifications or functional equivalents; used to obtain patents or other IP claiming use of the product; used to develop, test, or manufacturer a commercial product; used as a component in a commercial product; resold or licensed; used in any clinical applications or trials; or used in humans. A license or limited commercial use agreement is required for use by any for-profit entity, use in services, and use in sponsored academic research. For information regarding any such use, please contact licensing@milliporesigma.com.

RESEARCH USE ONLY. This product is regulated in France when intended to be used for scientific purposes, including for import and export activities (Article L 1211-1 paragraph 2 of the Public Health Code). The purchaser (i.e. enduser) is required to obtain an import authorization from the France Ministry of Research referred in the Article L1245-5-1 II. of Public Health Code. By ordering this product, you are confirming that you have obtained the proper import authorization.

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.