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).
Taschner, A, Vilardo, E, Rossmanith, W, Holzmann, J, Buzet, A, Nachbagauer, C
Vilardo, E, Rossmanith, W
tRNA (guanine-N1-)-methyltransferase activity of mtRNase P [mitochondrial matrix]
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