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Predicted secondary structure and sequence conservation of RNase_MRP
Symbol RNase_MRP
Rfam RF00030
Other data
RNA type Gene; ribozyme
Domain(s) Eukaryota
GO 0006364 0000171 0000172
SO 0000385

RNase MRP (also called RMRP) is an enzymatically active ribonucleoprotein with two distinct roles in eukaryotes. In mitochondria it plays a direct role in the initiation of mitochondrial DNA replication. In the nucleus it is involved in precursor rRNA processing, where it cleaves the internal transcribed spacer 1 between 18S and 5.8S rRNAs. Despite distinct functions, RNase MRP has been shown to be evolutionarily related to RNase P. Like eukaryotic RNase P, RNase MRP is not catalytically active without associated protein subunits.

Mutations in the RNA component of RNase MRP cause cartilage-hair hypoplasia, a pleiotropic human disease.


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