Article
Queuine ameliorates impaired mitochondrial function caused by mt-tRNAAsn variants.
Journal of translational medicine - 22 Aug 2024
Lin Yan, Wang Jiayin, Zhuang Xingyu, Zhao Ying, Wang Wei, Wang Dongdong, Zhao Yuying, Yan Chuanzhu, Ji Kunqian
Abstract excerpt
BACKGROUND: Mitochondrial tRNA (mt-tRNA) variants have been found to cause disease. Post-transcriptional queuosine (Q) modifications of mt-tRNA can promote efficient mitochondrial mRNA translation. Q modifications of mt-tRNAAsn have recently been identified. Here, the therapeutic effectiveness of queuine was investigated in cells from patients with mt-tRNAAsn variants. METHODS: Six patients (from four families)...
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