Cloned (Comment) | Organism |
---|---|
transient expression of C-terminally Flag-tagged NSUN3 in HeLa cells, immunohistochemic analysis shows that NSUN3 predominantly localized in mitochondria | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | generation of NSUN3 knockout cells. When the knockout strain is rescued by plasmid-encoded NSUN3, f5C34 in mt-tRNAMet is partially restored | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | no obvious mitochondrial-targeting sequence is present in the N-terminal region of NSUN3, immunohistochemic analysis shows that NSUN3 predominantly localized in mitochondria | Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
S-adenosyl-L-methionine + cytidine34 in mitochondrial tRNA | Homo sapiens | - |
S-adenosyl-L-homocysteine + 5-methylcytidine34 in mitochondrial tRNA | - |
? | |
S-adenosyl-L-methionine + cytosine34 in mitochondrial tRNAMet(AUA) precursor | Homo sapiens | - |
S-adenosyl-L-homocysteine + 5-methylcytosine34 in mitochondrial tRNAMet(AUA) precursor | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q9H649 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
HeLa cell | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
S-adenosyl-L-methionine + cytidine34 in mitochondrial tRNA | - |
Homo sapiens | S-adenosyl-L-homocysteine + 5-methylcytidine34 in mitochondrial tRNA | - |
? | |
S-adenosyl-L-methionine + cytosine34 in mitochondrial tRNAMet(AUA) precursor | - |
Homo sapiens | S-adenosyl-L-homocysteine + 5-methylcytosine34 in mitochondrial tRNAMet(AUA) precursor | - |
? | |
S-adenosyl-L-methionine + cytosine34 in mitochondrial tRNAMet(AUA) precursor | secondary structure of human mt-tRNAMet with modifications f5C and pseudouridine. The anticodon pairs with AUG and AUA codons, overview | Homo sapiens | S-adenosyl-L-homocysteine + 5-methylcytosine34 in mitochondrial tRNAMet(AUA) precursor | - |
? |
Synonyms | Comment | Organism |
---|---|---|
NSUN3 | - |
Homo sapiens |
NSUN3 methylase | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
S-adenosyl-L-methionine | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | NSUN3-knockout cells show strong reduction in mitochondrial protein synthesis and reduced oxygen consumption, leading to deficient mitochondrial activity. Reconstitution of formation of 5-methylcytidine (m5C) at position 34 (m5C34) on mt-tRNAMet with recombinant NSUN3 in the presence of AdoMet. Two disease-associated point mutations in mt-tRNAMet that impair m5C34 formation by NSUN3, are determined, indicating that a lack of f5C34 has pathological consequences. Loss of NSUN3 causes mitochondrial dysfunction, phenotype, overview | Homo sapiens |
physiological function | in human mitochondria, the AUA codon encodes methionine via a mitochondrial transfer RNA for methionine (mt-tRNAMet) that contains 5-formylcytidine (f5C) at the first position of the anticodon (position 34). f5C34 is required for deciphering the AUA codon during protein synthesis. Biogenesis of f5C34 is initiated by S-adenosylmethionine (AdoMet)-dependent methylation catalyzed by NSUN3, a methyltransferase in mitochondria. NSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNAMet. NSUN3 is essential for f5C34 formation | Homo sapiens |