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GAR1

Domain

Interaction with SMN1 requires at least one of the RGG-box regions.

Function

Required for ribosome biogenesis and telomere maintenance. Part of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. May also be required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme.

Sequence Similarities

Belongs to the GAR1 family.

Cellular localization

Alternative names

NOLA1, GAR1, H/ACA ribonucleoprotein complex subunit 1, Nucleolar protein family A member 1, snoRNP protein GAR1

swissprot:Q9NY12 entrezGene:54433 omim:606468