Target intelligence / Profile preview

tRNA isopentenyltransferase 1 (TRIT1)

Target
TRIT1
Molecular classification
Enzyme
01

Overview

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.

Other names
MOD5tRNA dimethylallyltransferaseIPTIPP transferaseIPPTIPTaseFLJ20061hGRO1COXPD35GRO1tRNA isopentenylpyrophosphate transferase
02

Biological functions

tRNA modification (specifically, addition of an isopentenyl group to adenine 37 of certain tRNAs)Protein translation fidelitySelenoprotein expressionStabilization of codon-anticodon interactions in translation
03

Disease associations

Neurodevelopmental disease (due to mitochondrial insufficiency)Mitochondrial disease (Combined oxidative phosphorylation deficiency 35)Cancer (tumor suppressor role)
04

Safety considerations

Pathogenic mutations can cause mitochondrial dysfunction and impaired protein translation, resulting in neurodevelopmental and mitochondrial disease

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