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tRNA isopentenyltransferase 1 (TRIT1) is an enzyme localized mainly to mitochondria and responsible for catalyzing the transfer of an isopentenyl group from dimethylallyl diphosphate (DMAPP) onto the adenine residue at position 37 of select tRNA molecules, forming N^6-(dimethylallyl)adenosine (i^6A37). This specific tRNA modification is essential for maintaining the proper reading frame and efficiency during protein translation, particularly stabilizing codon-anticodon base pairing for tRNAs that decode codons starting with uridine. TRIT1 modifies cytosolic and mitochondrial tRNAs, with a key role in selenoprotein expression and mitochondrial mRNA translation. Mutations causing loss of TRIT1 function can lead to combined oxidative phosphorylation deficiency and manifest as neurodevelopmental disorders due to impaired mitochondrial translation and function. TRIT1 has been described as a tumor suppressor, with its expression potentially reducing cell proliferation.
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