Reactome | PUS3 transforms uridine-39 to pseudouridine-39 in tRNA

PUS3 transforms uridine-39 to pseudouridine-39 in tRNA

Stable Identifier

R-HSA-8870289

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

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PUS3 transforms uridine-39 to pseudouridine-39 in tRNA

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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