The human tRNA taurine modification enzyme GTPBP3 is an active GTPase linked to mitochondrial diseases
نویسندگان
چکیده
منابع مشابه
Characterization of human GTPBP3, a GTP-binding protein involved in mitochondrial tRNA modification.
Human GTPBP3 is an evolutionarily conserved, multidomain protein involved in mitochondrial tRNA modification. Characterization of its biochemical properties and the phenotype conferred by GTPBP3 inactivation is crucial to understanding the role of this protein in tRNA maturation and its effects on mitochondrial respiration. We show that the two most abundant GTPBP3 isoforms exhibit moderate aff...
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M itochondria are integral to proper cell function, and mutations in its small genome (mtDNA) are associated with many diseases, along with the progression of normal aging [9]. While mtDNA has been extensively studied, not much is known about transcriptional variations of mitochondrial genes [3]. Recently, tRNA modifications have been the focus of intense study owing to their putative role in d...
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Modified uridine containing taurine, 5-taurinomethyluridine (τm5U), is found at the anticodon first position of mitochondrial (mt-)transfer RNAs (tRNAs). Previously, we reported that τm5U is absent in mt-tRNAs with pathogenic mutations associated with mitochondrial diseases. However, biogenesis and physiological role of τm5U remained elusive. Here, we elucidated τm5U biogenesis by confirming th...
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GTPBP3 is an evolutionary conserved protein presumably involved in mitochondrial tRNA (mt-tRNA) modification. In humans, GTPBP3 mutations cause hypertrophic cardiomyopathy with lactic acidosis, and have been associated with a defect in mitochondrial translation, yet the pathomechanism remains unclear. Here we use a GTPBP3 stable-silencing model (shGTPBP3 cells) for a further characterization of...
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ژورنال
عنوان ژورنال: Nucleic Acids Research
سال: 2021
ISSN: 0305-1048,1362-4962
DOI: 10.1093/nar/gkab104