TRMT10C:HSD17B10 (TRMT10C:SDR5C1) of mitochondrial RNase P methylates guanosine-9 in tRNA yielding 1-methylguanosine-9

Stable Identifier
Reaction [transition]
Homo sapiens
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TRMT10C in TRMT10C:HSD17B10 (TRMT10C:SDR5C1), a subcomplex of the mitochondrial RNase P complex, methylates the 1 position of guanosine-9 in mitochondrial tRNAs (Vilardo et al. 2012). 5 of 22 mitochondrial tRNAs have a G9 residue. Methylation of G9 appears to be important for correct folding of tRNA. Mutations in the SDR5C1 dehydrogenase subunit of RNase P impair dehydrogenation, tRNA methylation, and tRNA processing, causing HSD10 disease, which is characterized by progressive neurodegeneration and cardiomyopathy (Vilardo and Rossmanith 2015).

Literature References
PubMed ID Title Journal Year
23042678 A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase--extensive moonlighting in mitochondrial tRNA biogenesis

Taschner, A, Vilardo, E, Rossmanith, W, Holzmann, J, Buzet, A, Nachbagauer, C

Nucleic Acids Res. 2012
25925575 Molecular insights into HSD10 disease: impact of SDR5C1 mutations on the human mitochondrial RNase P complex

Vilardo, E, Rossmanith, W

Nucleic Acids Res. 2015
Event Information
Catalyst Activity

tRNA (guanine-N1-)-methyltransferase activity of mtRNase P [mitochondrial matrix]

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