Mitochondrial complex II mutated SDHD cell line
Invented by Abey Bandara at Virginia Tech
- Datasheet
- References (1)
- Inventor Info
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 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 |
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. |
