TRMT10C

This gene encodes the precursor of a subunit of the mitochondrial ribonuclease P, which is involved in 5' processing of mitochondrial tRNAs. The encoded protein may confer RNA-binding capacity to mitochondrial ribonuclease P and may be essential for transcript processing, RNA modification, translation and mitochondrial respiration. [provided by RefSeq, Nov 2012]

Full Name

TRMT10C Gene(Protein Coding)TRNA Methyltransferase 10C, Mitochondrial RNase P Subunit

Function

Mitochondrial tRNA N1-methyltransferase involved in mitochondrial tRNA maturation (PubMed:18984158, PubMed:21593607, PubMed:23042678, PubMed:27132592).
Component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and PRORP/MRPP3, which cleaves tRNA molecules in their 5'-ends (PubMed:18984158).
Together with HSD17B10/MRPP2, forms a subcomplex of the mitochondrial ribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functions that are independent of the ribonuclease P activity (PubMed:23042678, PubMed:29040705).
The MRPP1-MRPP2 subcomplex catalyzes the formation of N1-methylguanine and N1-methyladenine at position 9 (m1G9 and m1A9, respectively) in tRNAs; TRMT10C/MRPP1 acting as the catalytic N1-methyltransferase subunit (PubMed:23042678).
The MRPP1-MRPP2 subcomplex also acts as a tRNA maturation platform: following 5'-end cleavage by the mitochondrial ribonuclease P complex, the MRPP1-MRPP2 subcomplex enhances the efficiency of 3'-processing catalyzed by ELAC2, retains the tRNA product after ELAC2 processing and presents the nascent tRNA to the mitochondrial CCA tRNA nucleotidyltransferase TRNT1 enzyme (PubMed:29040705).
In addition to tRNA N1-methyltransferase activity, TRMT10C/MRPP1 also acts as a mRNA N1-methyltransferase by mediating methylation of adenosine residues at the N1 position of MT-ND5 mRNA (PubMed:29072297).
Associates with mitochondrial DNA complexes at the nucleoids to initiate RNA processing and ribosome assembly.

Biological Process

Biological Process mitochondrial RNA 5'-end processing Source:UniProtKB1 Publication
Biological Process mitochondrial tRNA 3'-end processing Source:UniProtKB1 Publication
Biological Process mitochondrial tRNA 5'-end processing Source:UniProtKB1 Publication
Biological Process mitochondrial tRNA methylation Source:ComplexPortal1 Publication
Biological Process mitochondrial tRNA processing Source:UniProtKB1 Publication
Biological Process mRNA methylation Source:UniProtKB1 Publication
Biological Process positive regulation of mitochondrial translation Source:UniProtKB1 Publication

Cellular Location

Mitochondrion matrix, mitochondrion nucleoid

Involvement in disease

Combined oxidative phosphorylation deficiency 30 (COXPD30):
An autosomal recessive, severe mitochondrial disease characterized by lactic acidosis, hypotonia, feeding difficulties, deafness, and respiratory failure with fatal issue. Patient skeletal muscle cells show decreased activities of mitochondrial complexes I, III and IV.