TRMT10C:HSD17B10 (TRMT10C:SDR5C1) methylates adenosine-9 in tRNA yielding 1-methyladenosine-9

Stable Identifier
Reaction [transition]
Homo sapiens
Locations in the PathwayBrowser
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TRMT10C of TRMT10C:HSD17B10 (TRMT10C:SDR5C1), a subcomplex of the mitochondrial RNase P complex, methylates the 1 position of adenosine-9 in mitochondrial tRNAs (Vilardo et al. 2012). 14 of 22 mitochondrial tRNAs have an A9 residue. Methylation of A9 appears to be important for correct folding of tRNA (Helm et al. 1998). Mutations in the HSD17B10 (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
9512533 The presence of modified nucleotides is required for cloverleaf folding of a human mitochondrial tRNA

Degoul, F, Florentz, C, Giegé, R, Brulé, H, Cepanec, C, Helm, M, Leroux, JP

Nucleic Acids Res. 1998
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 (adenine(9)-N1)-methyltransferase activity of mtRNase P [mitochondrial matrix]

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