Deletion of Gtpbp3 in zebrafish revealed the hypertrophic cardiomyopathy manifested by aberrant mitochondrial tRNA metabolism

GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of τm5U at the wobble position of mitochondrial tRNAGlu, tRNAGln, tRNALys, tRNATrp and tRNALeu(UUR). The previous investigations showed that GTPBP3 mutations were associated with hypertrophic cardiomyopathy (HCM). However, the p...

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Veröffentlicht in:	Nucleic acids research 2019-06, Vol.47 (10), p.5341-5355
Hauptverfasser:	Chen, Danni, Zhang, Zengming, Chen, Chao, Yao, Shihao, Yang, Qingxian, Li, Feng, He, Xiao, Ai, Cheng, Wang, Meng, Guan, Min-Xin
Format:	Artikel
Sprache:	eng
Schlagworte:	Aminoacylation Animals Cardiomyopathy, Hypertrophic - genetics Cardiomyopathy, Hypertrophic - metabolism CRISPR-Cas Systems Gene Deletion GTP-Binding Proteins - genetics GTP-Binding Proteins - metabolism In Situ Hybridization Mitochondria - genetics Mitochondria - metabolism Mitochondrial Proteins - metabolism Mutation Myocytes, Cardiac - metabolism Phenotype Protein Conformation RNA and RNA-protein complexes RNA, Transfer - metabolism Transgenes Zebrafish - genetics Zebrafish Proteins - genetics Zebrafish Proteins - metabolism
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