THG1L


Description

The THG1L (tRNA-histidine guanylyltransferase 1 like) is a protein-coding gene located on chromosome 5.

THG1L, the human gene encoding probable tRNA(His) guanylyltransferase, is an enzyme involved in tRNA modification and mitochondrial function.

THG1L catalyzes the addition of a guanosine monophosphate (GMP) to the 5'-end of tRNA(His) following transcription and RNase P cleavage. This modification is crucial for proper tRNA recognition and accurate protein synthesis. THG1L also acts as a guanyl-nucleotide exchange factor (GEF) for the mitofusins MFN1 and MFN2, thereby regulating mitochondrial fusion. By controlling both mitochondrial dynamics and energy production, THG1L promotes cell survival under oxidative stress.

THG1L is also known as ICF45, IHG-1, IHG1, SCAR28, THG1, hTHG1.

Associated Diseases


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