Engineering TALE-linked deaminases to facilitate precision adenine base editing in mitochondrial DNA

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70

초록

DddA-derived cytosine base editors (DdCBEs) and transcription activator -like effector (TALE) -linked deaminases (TALEDs) catalyze targeted base editing of mitochondrial DNA (mtDNA) in eukaryotic cells, a method useful for modeling of mitochondrial genetic disorders and developing novel therapeutic modalities. Here, we report that A -to -G -editing TALEDs but not C -to -T -editing DdCBEs induce tens of thousands of transcriptome-wide off -target edits in human cells. To avoid these unwanted RNA edits, we engineered the substrate -binding site in TadA8e, the deoxy-adenine deaminase in TALEDs, and created TALED variants with fine-tuned deaminase activity. Our engineered TALED variants not only reduced RNA off -target edits by >99% but also minimized off -target mtDNA mutations and bystander edits at a target site. Unlike wildtype versions, our TALED variants were not cytotoxic and did not cause developmental arrest of mouse embryos. As a result, we obtained mice with pathogenic mtDNA mutations, associated with Leigh syndrome, which showed reduced heart rates.

키워드

CRISPR-adenine base editor; genetic disease; in vivo genome editing; Leigh syndrome; mitochondria; mitochondrial genome editing; mtDNA; RNA off-target; TALE-linked adenine deaminase; TALED; RNA OFF-TARGET; STEM-CELLS; MUTATIONS; EDITORS; NUCLEAR
제목
Engineering TALE-linked deaminases to facilitate precision adenine base editing in mitochondrial DNA
저자
Cho, Sung-Ik; Lim, Kayeong; Hong, Seongho; Lee, Jaesuk; Kim, Annie; Lim, Chae Jin; Ryou, Seungmin; Lee, Ji Min; Mok, Young Geun; Chung, Eugene; Kim, Sanghun; Han, Seunghun; Cho, Sang-Mi; Kim, Jieun; Kim, Eun-Kyoung; Nam, Ki-Hoan; Oh, Yeji; Choi, Minkyung; An, Tae Hyeon; Oh, Kyoung-Ji; Lee, Seonghyun; Lee, Hyunji; Kim, Jin-Soo
DOI
10.1016/j.cell.2023.11.035
발행일
2024-01
유형
Article
저널명
Cell
권
187
호
1
페이지
95 ~ 109.e26