Mitochondrial complex II mutated SDHD cell line

The HEK293ΔSDHD 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.

Specific disruptions in SDHD have been reported to present clinically as the following disease conditions:
-Complex II deficiency
-Paraganglioma

 

This cell line is available from Ximbio; additional information and ordering details can be found here.

Patent Information:
For Information, Contact:
Rozzy Finn
Licensing Officer
Virginia Tech Intellectual Properties, Inc.
Rozzy@vt.edu
Inventors:
Aloka Bandara
Joshua Drake
David Brown
Keywords: