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Literature summary for 2.1.1.203 extracted from

  • Nakano, S.; Suzuki, T.; Kawarada, L.; Iwata, H.; Asano, K.; Suzuki, T.
    NSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNAMet (2016), Nat. Chem. Biol., 12, 546-551 .
    View publication on PubMed
Search for more literature for 2.1.1.203

Cloned(Commentary)

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

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

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
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Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
S-adenosyl-L-methionine + cytidine34 in mitochondrial tRNA Homo sapiens
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 S-adenosyl-L-homocysteine + 5-methylcytidine34 in mitochondrial tRNA
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 ?
S-adenosyl-L-methionine + cytosine34 in mitochondrial tRNAMet(AUA) precursor Homo sapiens
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 S-adenosyl-L-homocysteine + 5-methylcytosine34 in mitochondrial tRNAMet(AUA) precursor
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 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens Q9H649
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Source Tissue

Source Tissue Comment Organism Textmining
HeLa cell
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 Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
S-adenosyl-L-methionine + cytidine34 in mitochondrial tRNA
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 Homo sapiens S-adenosyl-L-homocysteine + 5-methylcytidine34 in mitochondrial tRNA
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 ?
S-adenosyl-L-methionine + cytosine34 in mitochondrial tRNAMet(AUA) precursor
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 Homo sapiens S-adenosyl-L-homocysteine + 5-methylcytosine34 in mitochondrial tRNAMet(AUA) precursor
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 ?
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
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 ?

Synonyms

Synonyms Comment Organism
NSUN3
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 Homo sapiens
NSUN3 methylase
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 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
S-adenosyl-L-methionine
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 Homo sapiens

General Information

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