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Biology subjects

Heckel, A.-M.

Publications and source records attributed to Heckel, A.-M..

2 recordsLinked to original sources

Modified crRNA variants for precise targeting of point mutations in human mitochondrial DNA by CRISPR-Cas12a

The CRISPR-Cas12a system, primarily used for nuclear genome editing, operates through RNA-guided cleavage of target DNA. Given the high copy number of mitochondrial DNA (mtDNA) in cells and the pathogenic potential of mtDNA mutations, there is substantial interest in developing methods to selectively eliminate mutant mtDNA without affecting wild-type molecules. We suggest using AsCas12a, with its shorter CRISPR RNA (crRNA) and proven ability to be targeted into human mitochondria, to perform selective cleavage of mutant mtDNA. Here, we assess the potential of AsCas12a and various engineered crRNAs to discriminate between wild-type and mutant mtDNA in vitro and in living human cells. By exploring chemically synthesized full and split crRNAs with various modifications, we identified crRNAs capable of targeting specific mutant mtDNA. We demonstrated the strong influence of magnesium concentration on the ability of crRNAs to discriminate point mutations. Finally, we show that AsCas12a-fused cytosine base editor can be addressed to mitochondria and can direct low but significant specific base editing of mtDNA in human cells. Our study suggests AsCas12a as a promising tool for targeted mutant mtDNA elimination or editing.

molecular biology↗

Targeted deletions in human mitochondrial DNA engineered by Type V CRISPR-Cas12a system

Mutations in mitochondrial DNA (mtDNA) contribute to various neuromuscular diseases, with severity depending on heteroplasmy level when mutant and wild-type mtDNA coexist within the same cell. Developing methods to model mtDNA dysfunction is crucial for experimental therapies. Here, we adapted the Type V CRISPR-AsCas12a system, which recognizes AT-rich PAM sequences, for targeted editing of human mtDNA. We show that AsCas12a effector, fused with a mitochondrial targeting sequence (MTS) from Neurospora crassa ATPase subunit 9, is efficiently addressed into human mitochondria and induces specific mtDNA cleavage in human cells. As a proof-of-concept, we demonstrate that AsCas12a, complexed with two crRNAs targeting distant regions of human mtDNA, introduces specific deletions in mtDNA. For the first time, we provide experimental data proving that a CRISPR system can be used not only for mtDNA degradation but also for precise mtDNA manipulation, offering a potential therapeutic avenue to address mitochondrial disorders.

molecular biology↗