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NSUN3
Mitochondrial tRNA methyltransferase that mediates methylation of cytosine to 5-methylcytosine (m5C) at position 34 of mt-tRNA(Met). mt-tRNA(Met) methylation at cytosine(34) takes place at the wobble position of the anticodon and initiates the formation of 5-formylcytosine (f(5)c) at this position. mt-tRNA(Met) containing the f(5)c modification at the wobble position enables recognition of the AUA codon in addition to the AUG codon, expanding codon recognition in mitochondrial translation. Belongs to the class I-like SAM-binding methyltransferase superfamily. RsmB/NOP family. Note: This description may include information from UniProtKB.
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| Protein type: EC 2.1.1.-; Methyltransferase |
| Chromosomal Location of human Ortholog: 3q11.2 |
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Cellular Component:
mitochondrial large ribosomal subunit; mitochondrial matrix; mitochondrion
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Molecular Function:
methyltransferase activity; tRNA (cytosine-5-)-methyltransferase activity; tRNA binding
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Biological Process:
regulation of mitochondrial translation; rRNA methylation; tRNA wobble base cytosine methylation
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Disease: Combined Oxidative Phosphorylation Deficiency 48
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Reference #:
Q9H649
(UniProtKB)
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Alt. Names/Synonyms: COXPD48; FLJ22109; FLJ22609; MST077; MSTP077; NOL1/NOP2/Sun domain family 3; NOL1/NOP2/Sun domain family member 3; NOL1/NOP2/Sun domain family, member 3; NOP2/Sun domain family, member 3; NOP2/Sun RNA methyltransferase 3; NOP2/Sun RNA methyltransferase family member 3; NSUN3; Putative methyltransferase NSUN3; tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial
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Gene Symbols: NSUN3
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Molecular weight:
38,244 Da
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Basal Isoelectric point:
9.09
Predict pI for various phosphorylation states
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