Reaction: TRMT10C:HSD17B10 (TRMT10C:SDR5C1) of mitochondrial RNase P methylates guanosine-9 in tRNA yielding 1-methylguanosine-9
- in pathway: tRNA modification in the mitochondrion
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).
Reaction - small molecule participants:
AdoHcy [mitochondrial matrix]
AdoMet [mitochondrial matrix]
Reactome.org reaction link: R-HSA-6787591
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Reaction input - small molecules:
S-adenosyl-L-methionine
Reaction output - small molecules:
S-adenosyl-L-homocysteine
Reactome.org link: R-HSA-6787591