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Ribonuclease MRP subunit p64 (RMP64)

Target
RMP64
Molecular classification
Other (nuclear ribonucleoprotein complex component), Enzyme subunit (RNase MRP complex), Not an isolated enzyme/receptor/transport protein/transcription factor
01

Overview

Ribonuclease MRP subunit p64 (RMP64) is a recently characterized, poorly understood protein component unique to the human RNase MRP ribonucleoprotein complex, an endoribonuclease essential for precursor ribosomal RNA (pre-rRNA) processing at the ITS1 site 2 in the nucleolus[1]. RMP64, together with another unique subunit (RMP24), is required for proper pre-rRNA maturation but is not necessary for RNase P activity and does not associate with the RNase P-specific H1 RNA, highlighting functional specialization[1]. It is predicted to participate in the regulation of neural progenitor differentiation and in the positive regulation of the Notch signaling pathway[2][3]. RMP64 is not an independent enzyme or receptor, nor is it a recognized therapeutic target, but rather a functional subunit of a non-coding ribonucleoprotein complex. Mutations impairing RNase MRP function (primarily through the RNA component) can lead to a spectrum of developmental and skeletal disorders, although specific roles of individual protein subunits, including RMP64, in human disease have not been defined[4].

Other names
Nucleolus and neural progenitor proteinNEPROC3orf17DKFZP434F2021NET17ANXD3protein nepro homolognucleolus and neural progenitor protein
02

Mechanism of action

No direct drug mechanism described or drugs known to target RMP64

03

Biological functions

Pre-rRNA processing at ITS1 site 2 (ribosomal RNA maturation)Negative regulation of neuron differentiationPositive regulation of Notch signaling pathwayAssociation with ribonucleoprotein enzyme involved in rRNA biogenesisNot required for RNase P activity, suggesting distinct roles from shared subunits
04

Disease associations

Other (no direct disease association for RMP64 as a subunit, but RNase MRP complex linked to skeletal dysplasias through its RNA component)RNase MRP complex mutations associated with: Cartilage–hair hypoplasiaMetaphyseal dysplasia without hypotrichosisAnauxetic dysplasiaKyphomelic dysplasiaOmenn syndromeThese are due to mutations in the RNA component, not specifically protein subunits such as RMP64
05

Safety considerations

None specific; not a therapeutic target or druggable protein
06

Interacting drugs

None known
07

Biomarkers

None established for RMP64; RNase MRP RNA may be of biomarker interest in certain syndromes

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