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HEK293ΔNDUFS2 Cell Line

Invented by Abey Bandara at Virginia Tech

Info

Catalogue Number 156497
Antigen/Gene or Protein Targets NDUFS2
Parental Line HEK293
Synonyms HEK293ΔNDUFS2
Host Human
Disease Keywords Leighs; Mitochondrial disease; cardiomyopathy; encephalomyopathy
Model Knock-Out
Relevance The HEK293ΔNDUFS2 cell line expresses a CRISPR/Cas9 mutated NDUFS2, shown to significantly alter the function of complex I (CI) of the mitochondrial electron transport chain. This NDUFS2 mutant cell line displays disruptions to NDUFS2 that mimic isolated complex I deficiency and can be used as a powerful model of several types of mitochondrial disease.

Specific disruptions in the NDUFS2 gene have been reported to present clinically as the following disease conditions:
-Leigh syndrome
-Cardiomyopathy
-Encephalomyopathy
-Miscellaneous: basal ganglia and brainstem lesions, seizures, hypotonia, neonatal hypotonia, amaurosis, nystagmus, dysmorphic features, epilepsy and signs of brainstem involvement, and lactic acidosis.
Production Details The cell line is currently stored at -80ºC at Virginia Tech. By culturing approximately 1 million frozen cells in growth media, approximately 40 million cells can be harvested in a two weeks time.
Research Area Apoptosis and Programmed Cell Death, Cardiovascular, Drug Discovery & Development, Metabolism
Growth/Phenotype Keywords The mutant grows much slower than the parent cell line. The doubling time of the mutant is 3 times higher than that of the parent cell line.
Recommended Growing Conditions DMEM medium supplemented with 10% Fetal Bovine Serum and 1% Penicillin-Streptomycin at 37ºC temperature and 5-6% CO2.
Positive Control HEK293 parent line
Notes *It is incumbent upon the recipient of this cell line to procure a license for the commercialization/commercial use of the CRISPR edited material contained herein. Ximbio does not currently hold a license to commercialize CRISPR based technologies.

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