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

Stable Identifier
R-HSA-6787594
Type
Reaction [transition]
Species
Homo sapiens
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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
23042678 A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase--extensive moonlighting in mitochondrial tRNA biogenesis

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

Nucleic Acids Res. 2012
9512533 The presence of modified nucleotides is required for cloverleaf folding of a human mitochondrial tRNA

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

Nucleic Acids Res. 1998
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
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Catalyst Activity
Title
tRNA (adenine-N1-)-methyltransferase activity of mtRNase P [mitochondrial matrix]
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