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Gene Family: mitochondrial RNase P complex

Also known as : "mitochondrial PRORP", "protein only RNase P", "proteinaceous RNase P", "mt-RNase P complex "

mitochondrial RNase P complex:: Metazoans have two RNase P enzymes: a nuclear RNA-based RNase P and a mitochondrial PRORP (protein-only RNase P). Human mitochondrial RNase P consists of three subunits and lacks an RNA component. RNase P enzymes are metal ion-dependent endonucleases: hydrolases that catalyze site-specific phosphodiester bond hydrolysis primarily within pre-tRNA. [Source: Klem et al, 2016]

Genes contained within the family: 3

Approved Symbol Approved Name Previous Symbols Synonyms Chromosome
TRMT10C tRNA methyltransferase 10C, mitochondrial RNase P subunit RG9MTD1 MRPP1, FLJ20432 3q12.3
HSD17B10 hydroxysteroid 17-beta dehydrogenase 10 HADH2, MRXS10 SDR5C1, ABAD, MRPP2, 17b-HSD10, ERAB, CAMR, MHBD Xp11.22
KIAA0391 KIAA0391 MRPP3, PRORP 14q13.2