Article
Ablation of Mto1 in zebrafish exhibited hypertrophic cardiomyopathy manifested by mitochondrion RNA maturation deficiency.
Nucleic acids research - 7 May 2021
Zhang Qinghai, He Xiao, Yao Shihao, Lin Tianxiang, Zhang Luwen, Chen Danni, Chen Chao, Yang Qingxian, Li Feng, Zhu Yi-Min, Guan Min-Xin
Abstract excerpt
Deficient maturations of mitochondrial transcripts are linked to clinical abnormalities but their pathophysiology remains elusive. Previous investigations showed that pathogenic variants in MTO1 for the biosynthesis of τm5U of tRNAGlu, tRNAGln, tRNALys, tRNATrp and tRNALeu(UUR) were associated with hypertrophic cardiomyopathy (HCM). Using mto1 knock-out(KO) zebrafish generated by CRISPR/Cas9 system, we...
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