Mitochondrial complex II mutated SDHD cell line

This cell line has been shown to significantly alter the function of complex II (CII) of the mitochondrial electron transport chain and displays disruptions to SDHD that mimic clinical presentations of CII deficiency.

Contributors

Inventor Institute
Abey Bandara Virginia Tech
Cat. #: 160706
Tool sub type: Continuous
Unit size: 1×10^6 cells / vial
Research Fields: Cancer;Drug development;Metabolism
Organism: Human
Model: Knock-Out
Growth properties: The mutant has been shown to grow much slower than the parental cell line. The doubling time of the mutant is also significantly slower than that of the parental line.
Alternate name: HEK293?SDHD
Product description: The HEK293?SDHD cell line expresses a CRISPR/Cas9 mutated nuclear encoded subunit, SDHD, of mitochondrial complex II (succinate dehydrogenase). This cell line has been shown to significantly alter the function of complex II (CII) of the mitochondrial electron transport chain and displays disruptions to SDHD that mimic clinical presentations of CII deficiency. These cells represent a powerful model of metabolic pathologies, not only for mitochondrial disease, but also for understanding the importance of CII in developing novel therapeutics. CRISPR edited HEK293 cells.Specific disruptions in SDHD have been reported to present clinically as the following disease conditions:-Complex II deficiency-Paraganglioma
Production details: The cell line is currently stored at Virginia Tech.
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 provi…
Parental cell line: HEK293
Disease: Mitochondrial Disease
Format: Frozen
Shipping conditions: Dry ice
Growth medium: DMEM medium supplemented with 10% Fetal Bovine Serum and 1% Penicillin-Streptomycin at 37?‚°C temperature and 5-6% CO2.
Recommended controls: HEK293 parental line
References:

Bandara et al. 2021. BMC Mol Cell Biol. 22(1):35. PMID: 34118887.

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