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

Stable Identifier
R-HSA-6787591
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
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
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
Participants
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
Title
tRNA (guanine-N1-)-methyltransferase activity of mtRNase P [mitochondrial matrix]
Physical Entity
Activity
Authored
Reviewed
Created