The COQ6 Knockout Raji Polyclonal Cells are a CRISPR/Cas9-edited polyclonal knockout cell population derived from the Raji B lymphocyte line, featuring targeted disruption of the COQ6 gene. This model enables loss-of-function studies of COQ6, a monooxygenase essential for ubiquinone biosynthesis, and provides a heterogeneous knockout pool ideal for population-level mitochondrial dysfunction analysis. Quality-controlled for robust gene disruption, it is intended for advanced biomedical research.
The Raji cell line is a suspension lymphoblastoid line from a Burkitt lymphoma patient, EBV-positive and widely used in B cell biology and lymphoma research. Its high proliferation rate depends on mitochondrial metabolism, making it suitable for investigating the metabolic consequences of ubiquinone deficiency. COQ6 knockout in this background dissects mitochondrial pathways in a lymphoma-relevant environment.
COQ6 functions as a monooxygenase catalyzing the C5-hydroxylation step of ubiquinone biosynthesis, interacting with complex components COQ5, COQ7, COQ9, and COQ8A. Its expression is regulated by NRF2 and PPARGC1A under oxidative stress. Disruption leads to CoQ10 deficiency, impairing mitochondrial electron transport chain complexes I-III, reducing ATP synthesis, and increasing ROS production. This triggers apoptosis signaling and compromises oxidative phosphorylation, with downstream effects on mitochondrial membrane potential and ubiquinone levels. Thus, COQ6 is a critical node connecting redox regulation and mitochondrial bioenergetics.
In Raji cells, COQ6 loss models primary CoQ10 deficiency, linked to nephrotic syndrome, hearing loss, and encephalopathy. The EBV-transformed B cell background enables study of ubiquinone deficiency in immune cell metabolism, with implications for B cell receptor signaling, proliferation, and lymphomagenesis. This model bridges metabolic defects and B cell pathology, offering insights into CoQ-dependent processes in lymphoma.
Researchers can use this model to investigate CoQ10 deficiency pathophysiology via ubiquinone HPLC quantification, OCR assays, ATP and ROS measurements. Confirm knockout with Western blot and RT-qPCR, and assess mitochondrial membrane potential (JC-1), apoptosis (Annexin V), and proliferation. It is suitable for screening CoQ biosynthesis modulators or evaluating CoQ10 rescue. Thus, it enables delineation of COQ6 roles in B lymphocyte metabolism and mitochondrial disease. For more information, contact Ascent Research.
