HEK293DeltaNDUFS2 Cell Line
- Name Abey Bandara
- Institute Virginia Tech
Tool name: HEK293DeltaNDUFS2 Cell Line
Alternate names: HEK293?NDUFS2
Tool type: Cell Lines
Tool sub-type: Continuous
Parental cell line: HEK293
Disease: Leighs; Mitochondrial disease; Cardiomyopathy; Encephalomyopathy
Growth properties: 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.
Description: The HEK293?NDUFS2 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:
-Miscellaneous: basal ganglia and brainstem lesions, seizures, hypotonia, neonatal hypotonia, amaurosis, nystagmus, dysmorphic features, epilepsy and signs of brainstem involvement, and lactic acidosis.
Research area: Apoptosis and autophagy; Drug development; Metabolism
Production details: The cell line is currently stored 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.
Additional notes: CRISPR edited HEK293 cells.
Cancer Research Technology Limited (trading research tools as Ximbio) has been granted a non-exclusive license to the CRISPR-Cas9 technology by ERS Genomics Ltd under the patent rights listed here.
This license from ERS Genomics Ltd allows Ximbio to develop and commercialise CRISPR-Cas9 modified cell lines for research use only. Ximbio can provide these modified CRISPR-Cas9 cell lines to companies under a label-use only license.
- For Research Use Only
- • Bandara et al. 2021. Mitochondrion. 58:160-168. PMID: 33744462.