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The human tRNA taurine modification enzyme GTPBP3 is an active GTPase linked to mitochondrial diseases.

Nucleic acids research - 18 Mar 2021

Peng Gui-Xin, Zhang Yong, Wang Qin-Qin, Li Qing-Run, Xu Hong, Wang En-Duo, Zhou Xiao-Long

Abstract excerpt

GTPBP3 and MTO1 cooperatively catalyze 5-taurinomethyluridine (τm5U) biosynthesis at the 34th wobble position of mitochondrial tRNAs. Mutations in tRNAs, GTPBP3 or MTO1, causing τm5U hypomodification, lead to various diseases. However, efficient in vitro reconstitution and mechanistic study of τm5U modification have been challenging, in part due to the lack of pure and active enzymes. A previous study reported...

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PMID: 33619562
DOI: 10.1093/nar/gkab104
PMCID: PMC7969015

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