NSUN3

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Description

The NSUN3 (NOP2/Sun RNA methyltransferase 3) is a protein-coding gene located on chromosome 3.

NSUN3 is a mitochondrial tRNA methyltransferase that modifies cytosine to 5-methylcytosine (m5C) at position 34 of mt-tRNA(Met). This methylation occurs at the wobble position of the anticodon and initiates the formation of 5-formylcytosine (f(5)c) at this position. The f(5)c modification allows mt-tRNA(Met) to recognize the AUA codon in addition to the AUG codon, expanding codon recognition during mitochondrial translation.

NSUN3 is also known as COXPD48, MST077, MSTP077.

Associated Diseases

• Combined oxidative phosphorylation deficiency 48