Reactome | RPUSD4 in the mitochondrial pseudouridylation module transforms uridine-1397 to pseudouridine-1397 in mitochondrial 16S rRNA

RPUSD4 in the mitochondrial pseudouridylation module transforms uridine-1397 to pseudouridine-1397 in mitochondrial 16S rRNA

Stable Identifier

R-HSA-9936243

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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RPUSD4 in the mitochondrial pseudouridylation module transforms uridine-1397 to pseudouridine-1397 in mitochondrial 16S rRNA

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RPUSD4 is part of a larger complex, the mitochondrial pseudouridylation module (FASTKD2:MTERF3:NGRN:RCC1L:RPUSD3:RPUSD4:TRUB2) (Arroyo et al. 2016, Antonicka et al. 2017), which binds 16S rRNA (Arroyo et al. 2016, Zaganelli et al. 2017, Antonicka et al. 2017), and cosediments with the mitochondrial large ribosomal subunit (Antonicka et al. 2017). RPUSD4 transforms a uridine residue at nucleotide 1397 of 16S rRNA to a pseudouridine residue (Zaganelli et al. 2017, Antonicka et al. 2017). Depletion of RPUSD4 causes a reduction in the amount of 16S rRNA (Arroyo et al. 2016, Zaganelli et al. 2017). Depletion of both RPUSD4 and TRUB2 by RNA interference causes a reduction in assembly of the mitochondrial large ribosomal subunit and the 55S ribosome (Antonicka et al. 2017).

Literature References

PubMed ID	Title	Journal	Year
27667664	A Genome-wide CRISPR Death Screen Identifies Genes Essential for Oxidative Phosphorylation Arroyo, JD, Jourdain, AA, Calvo, SE, Ballarano, CA, Doench, JG, Root, DE, Mootha, VK	Cell Metab	2016
28082677	The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules Zaganelli, S, Rebelo-Guiomar, P, Maundrell, K, Rozanska, A, Pierredon, S, Powell, CA, Jourdain, AA, Hulo, N, Lightowlers, RN, Chrzanowska-Lightowlers, ZM, Minczuk, M, Martinou, JC	J Biol Chem	2017
27974379	A pseudouridine synthase module is essential for mitochondrial protein synthesis and cell viability Antonicka, H, Choquet, K, Lin, ZY, Gingras, AC, Kleinman, CL, Shoubridge, EA	EMBO Rep	2017