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Stable nuclear expression of ATP8 and ATP6 genes rescues a mtDNA Complex V null mutant
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Simultaneous allotopic expression of the minimally recoded ATP6 and the codon-optimized ATP8 can rescue a patient-derived cybrid model null for the ATP8 protein.
“and demonstrated how simultaneous allotopic expression of two mitochondrial genes, the minimally recoded ATP6 and the codon-optimized ATP8, can rescue a patient-derived cybrid model null for the ATP8 protein.27”
From Exogenous expression of ATP8, a mitochondrial encoded protein, from the nucleus in vivo (2024) · cites this paper as [27]
Allotopic expression of ATP6 in patient cybrids with specific mutations such as mt.8993T>G or mt.8529G>A shows promise and can restore partial function.
“Allotopic expression of ATP6 in patient cybrids with specific mutations such as the mt.8993T>G or (mt.8529G>A) shows promise and can restore partial function.27,29,40”
From Exogenous expression of ATP8, a mitochondrial encoded protein, from the nucleus in vivo (2024) · cites this paper as [27]
Previously, only a fraction of allotopically expressed mitochondrial protein integrates into the proper mitochondrial complex.
“Previously, we have shown how only a fraction of allotopically expressed mitochondrial protein integrates into the proper mitochondrial complex.27,33”
From Exogenous expression of ATP8, a mitochondrial encoded protein, from the nucleus in vivo (2024) · cites this paper as [27]
The A8/A6mut cells were selected for homoplasmy as described previously.
“The A8/A6mut cells were selected for homoplasmy as described previously [87].”
From Codon optimization is an essential parameter for the efficient allotopic expression of mtDNA genes (2020) · cites this paper as [87]
The authors previously reported successful allotopic expression of MT-ATP8 and, to a lesser extent, MT-ATP6, with functional rescue in the A8/A6mut cybrid null for ATP8 protein.
“We previously reported the successful allotopic expression of the MT-ATP8 gene and to a lesser extent the MT-ATP6 gene, with functional rescue in a cybrid cell line A8/A6mut (m.8529G-A) null (Fig. 5A) for the ATP8 protein [87].”
From Codon optimization is an essential parameter for the efficient allotopic expression of mtDNA genes (2020) · cites this paper as [87]
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Chin, R. M., Panavas, T., Brown, J. M., & Johnson, K. K. (2018) · Cell Reports · doi:10.1016/j.celrep.2018.02.059 · Cited by 50
Codon optimization is an essential parameter for the efficient allotopic expression of mtDNA genes
Lewis, C. J., Dixit, B., Batiuk, E., Hall, C. J., O'Connor, M. S., & Boominathan, A. (2020) · Redox Biology · doi:10.1016/j.redox.2020.101429 · Cited by 20
Self-citationThe Mitochondrial Genome in Aging and Disease and the Future of Mitochondrial Therapeutics
Saravanan, S., Lewis, C. J., Dixit, B., O’Connor, M. S., Stolzing, A., & Boominathan, A. (2022) · Biomedicines · doi:10.3390/biomedicines10020490 · Cited by 13
Self-citationExogenous expression of ATP8, a mitochondrial encoded protein, from the nucleus in vivo
Begelman, D. V., Dixit, B., Truong, C., King, C. D., Watson, M. A., Schilling, B., Brand, M. D., & Boominathan, A. (2024) · Molecular Therapy - Methods & Clinical Development · doi:10.1016/j.omtm.2024.101372 · Cited by 5
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Metadata
- Authors
- Boominathan, A., Vanhoozer, S., Basisty, N., Powers, K., Crampton, A. L., Wang, X., Friedricks, N., Schilling, B., Brand, M. D., & O'Connor, M. S.
- Venue
- Nucleic Acids Research
- Publisher
- Oxford University Press (OUP)
- Published
- 2016-09-04
- Pages
- gkw756
- ISSN
- 0305-1048
- 1362-4962
- PMC
- PMC5100594
- Cited by
- 27
- References
- 4
- Language
- en
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