PUS3 transforms uridine-39 to pseudouridine-39 in tRNA

Stable Identifier
R-HSA-8870289
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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PUS3 catalyzes the modification (isomerization) of uridine to pseudouridine at nucleotides 38 and 39 in the anticodon loops of tRNAs (Shaheen et al. 2016). Homologues of PUS3 in mouse (Chen and Patton 2000) and yeast (Lecointe et al. 1998) catalyze the same reaction, and both the PUS3 gene family and the modification are highly conserved. Deletion of DEG1 encoding Pus3p in budding yeast (Saccharomyces cerevisiae) causes slow growth (Lecointe et al. 1998) while a truncating mutation in human PUS3 causes a reduction in pseudouridines 38 and 39 with consequent intellectual disability (Shaheen et al. 2016).

Literature References
PubMed ID Title Journal Year
27055666 A homozygous truncating mutation in PUS3 expands the role of tRNA modification in normal cognition

Shaheen, R, Han, L, Faqeih, E, Ewida, N, Alobeid, E, Phizicky, EM, Alkuraya, FS

Hum. Genet. 2016
9430663 Characterization of yeast protein Deg1 as pseudouridine synthase (Pus3) catalyzing the formation of psi 38 and psi 39 in tRNA anticodon loop

Lecointe, F, Simos, G, Sauer, A, Hurt, EC, Motorin, Y, Grosjean, H

J. Biol. Chem. 1998
11027153 Pseudouridine synthase 3 from mouse modifies the anticodon loop of tRNA

Chen, J, Patton, JR

Biochemistry 2000
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Catalyst Activity
Catalyst Activity
Title
pseudouridine synthase activity of PUS3 [cytosol]
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