tRNA-histidine guanylyltransferase 1 like (THG1L)

tRNA-histidine guanylyltransferase 1 like (THG1L) is a highly conserved mitochondrial enzyme crucial for the post-transcriptional modification of tRNA^His in humans, where it catalyzes the unusual 3′–5′ addition of a guanosine residue to the 5′ end of tRNA^His, a modification necessary for proper aminoacylation and accurate protein synthesis[2][6]. THG1L also acts as a guanyl-nucleotide exchange factor for mitochondrial fusion proteins MFN1 and MFN2, impacting mitochondrial dynamics and stress responses[2]. In disease, it is upregulated by high glucose and associated with mitochondrial biogenesis and fibrosis, notably in diabetic nephropathy, but its broader physiological and pathological roles remain under active study[4][7]. No small molecules or drugs have been described to target THG1L directly.

Other names

ICF45IHG-1IHG1SCAR28THG1hTHG1FLJ11601FLJ20546induced in high glucose-1Interphase cytoplasmic foci protein 45probable tRNA(His) guanylyltransferaseputative tRNA(His) guanylyltransferase