MRM2

The protein encoded by this gene is a member of the S-adenosylmethionine-binding protein family. It is a nucleolar protein and it may be involved in the processing and modification of rRNA. This gene has been suggested to be involved in cell cycle control and DNA repair.

Full Name

Mitochondrial RRNA Methyltransferase 2

Function

S-adenosyl-L-methionine-dependent 2'-O-ribose methyltransferase that catalyzes the formation of 2'-O-methyluridine at position 1369 (Um1369) in the 16S mitochondrial large subunit ribosomal RNA (mtLSU rRNA), a universally conserved modification in the peptidyl transferase domain of the mtLSU rRNA.

Biological Process

RNA methylation Source: GO_Central
rRNA 2'-O-methylation Source: Reactome
rRNA methylation Source: BHF-UCL
rRNA processing Source: BHF-UCL

Cellular Location

Mitochondrion

Involvement in disease

Mitochondrial DNA depletion syndrome 17 (MTDPS17):
An autosomal recessive mitochondrial disorder characterized by childhood onset of rapidly progressive encephalopathy, stroke-like episodes, lactic acidosis, hypocitrullinemia, multiple defects of oxidative phosphorylation, mitochondrial complex I and IV deficiency, and reduced mtDNA copy number.