HEK293ΔNDUFS2 Cell Line

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
-Miscellaneous: basal ganglia and brainstem lesions, seizures, hypotonia, neonatal hypotonia, amaurosis, nystagmus, dysmorphic features, epilepsy and signs of brainstem involvement, and lactic acidosis.


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.
Aloka Bandara
David Brown