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
mitochondrial matrix
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TRMT10C:HSD17B10 (TRMT10C:SDR5C1) of mitochondrial RNase P methylates guanosine-9 in tRNA yielding 1-methylguanosine-9
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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
Input
Output
Participates
as an event of
Event Information
Go Biological Process
mitochondrial tRNA methylation (0070901)
Catalyst Activity

tRNA (guanine-N1-)-methyltransferase activity of mtRNase P [mitochondrial matrix]

Physical Entity
mtRNase P [mitochondrial matrix] (Homo sapiens)
Activity
tRNA (guanine-N1-)-methyltransferase activity (GO:0009019)
Authored
May, B  (2015-07-14)
Reviewed
Levinger, L  (2015-08-11)
Motorin, Y  (2015-08-25)
Jarrous, N  (2015-10-24)
Powell, CA  (2015-11-07)
Created
May, B  (2015-07-15)
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