RNA component of mitochondrial RNA processing endoribonuclease (RMRP)

The RNA component of mitochondrial RNA processing endoribonuclease (RMRP) is a long non-coding RNA essential for cellular function[1][2][3]. It forms part of the RNase MRP ribonucleoprotein complex, which is involved in cleaving precursor ribosomal RNA (pre-rRNA) for ribosome biogenesis, and also processes mitochondrial RNA at sites critical for DNA replication[1][2][3]. RMRP interacts with telomerase reverse transcriptase to create an RNA-dependent RNA polymerase complex that generates double-stranded RNA processed into small interfering RNAs[1]. Pathogenic mutations in RMRP cause cartilage-hair hypoplasia, an inherited disorder marked by skeletal dysplasia, immunodeficiency, and cancer susceptibility[2][3]. RMRP is also aberrantly upregulated in several cancers, where it can promote proliferation and migration, partly through regulation of the AKT signaling pathway and by serving as a molecular sponge for certain tumor-suppressive microRNAs such as miR-206[4][5]. Though fundamental for RNA processing, RMRP is not a typical therapeutic target such as an enzyme or receptor, and there are currently no drugs known to directly target RMRP[1][2][3][4][5].

Other names

RMRPRRRP2NME1RNase MRP RNACHH