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RMRP RNA component of mitochondrial RNA processing endoribonuclease

Also known as: CHH; NME1; RRP2; RMRPR


This gene encodes the RNA component of mitochondrial RNA processing endoribonuclease, which cleaves mitochondrial RNA at a priming site of mitochondrial DNA replication. This RNA also interacts with the telomerase reverse transcriptase catalytic subunit to form a distinct ribonucleoprotein complex that has RNA-dependent RNA polymerase activity and produces double-stranded RNAs that can be processed into small interfering RNA. Mutations in this gene are associated with cartilage-hair hypoplasia.[provided by RefSeq, Mar 2010]

Genomic context

Chromosome: 9; NC_000009.12 (35657751..35658018, complement)
Total number of exons:


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