143B m.8993T>G isogenic mTUNE mT80 Cell Line
- Name Payam Gammage
- Institute Cancer Research UK, Glasgow: The Beatson Institute
Tool name: 143B m.8993T>G isogenic mTUNE mT80 Cell Line
Alternate names: mTUNE 8, mT8
Tool type: Cell Lines
Tool sub-type: Continuous
Parental cell line: 143B human osteosarcoma (m.8993T>G cybrid)
Cancer type: Bone cancer, Osteosarcoma
Description: The cellular metabolic environemnt is influenced by relative levels of coexistant mutant and wild-type mitochondrial DNAs (heteorplasmy) in a range of diseases for which mitochondrial dysfunction is a feature. Disease phenotypes may be rescued by returning heteroplasmy towards wild-type levels.
To investigate this approach, the team led by Payam Gammage have devised a series of cell lines which stably express stable levels of mutatnt m.8993T>G mtDNA heteroplasmy.
Cell lines bearing m.8993T>G heteroplasmy of 80, 45, and 10% are available at Ximbio.
These lines are derivatives of the m.8993T cybrid cell developed by Prof Eric Schon, and are of of 143B human osteosarcoma lineage.
Research area: Cancer ; Metabolism
- For Research Use Only
- • Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo.
- • Enhanced Manipulation of Human Mitochondrial DNA Heteroplasmy In Vitro Using Tunable mtZFN Technology.
- • NADH Shuttling Couples Cytosolic Reductive Carboxylation of Glutamine with Glycolysis in Cells with Mitochondrial Dysfunction.
- • Engineered mtZFNs for Manipulation of Human Mitochondrial DNA Heteroplasmy.
- • Near-complete elimination of mutant mtDNA by iterative or dynamic dose-controlled treatment with mtZFNs.
- • Mitochondrially targeted ZFNs for selective degradation of pathogenic mitochondrial genomes bearing large-scale deletions or point mutations.