Aliases for QTRT1 Gene
External Ids for QTRT1 Gene
Previous GeneCards Identifiers for QTRT1 Gene
This gene encodes the catalytic subunit of tRNA-guanine transglycosylase. tRNA-guanine transglycosylase is a heterodimeric enzyme complex that plays a critical role in tRNA modification by synthesizing the 7-deazaguanosine queuosine, which is found in tRNAs that code for asparagine, aspartic acid, histidine and tyrosine. A pseudogene of this gene is located on the long arm of chromosome X. [provided by RefSeq, Feb 2012]
GeneCards Summary for QTRT1 Gene
QTRT1 (Queuine TRNA-Ribosyltransferase Catalytic Subunit 1) is a Protein Coding gene. Diseases associated with QTRT1 include Atypical Autism and Benign Epilepsy With Centrotemporal Spikes. Among its related pathways are Gene Expression and tRNA processing. Gene Ontology (GO) annotations related to this gene include queuine tRNA-ribosyltransferase activity. An important paralog of this gene is QTRT2.
UniProtKB/Swiss-Prot Summary for QTRT1 Gene
Catalytic subunit of the queuine tRNA-ribosyltransferase (TGT) that catalyzes the base-exchange of a guanine (G) residue with queuine (Q) at position 34 (anticodon wobble position) in tRNAs with GU(N) anticodons (tRNA-Asp, -Asn, -His and -Tyr), resulting in the hypermodified nucleoside queuosine (7-(((4,5-cis-dihydroxy-2-cyclopenten-1-yl)amino)methyl)-7-deazaguanosine) (PubMed:11255023, PubMed:20354154). Catalysis occurs through a double-displacement mechanism. The nucleophile active site attacks the C1' of nucleotide 34 to detach the guanine base from the RNA, forming a covalent enzyme-RNA intermediate. The proton acceptor active site deprotonates the incoming queuine, allowing a nucleophilic attack on the C1' of the ribose to form the product (By similarity).