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RNA binding region (RNP1, RRM) containing 3 (RNPC3)

Target
RNPC3
Molecular classification
Spliceosomal protein (component of minor spliceosome), RNA-binding protein (contains RNA recognition motifs; RRM), Other: RNA processing factor
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Overview

RNA binding region (RNP1, RRM) containing 3 (RNPC3) is a protein component of the minor spliceosome, which specifically catalyzes the removal of U12-type introns from pre-mRNA, a process required for the maturation of specific mRNA transcripts. RNPC3 contains two RNA recognition motifs, allowing it to bind U12 snRNA and promote the formation of the U11/U12 ribonucleoprotein complex. It has been identified as an autoantigen in certain autoimmune diseases, notably systemic sclerosis and mixed connective tissue disease, and anti-RNPC3 autoantibodies can serve as biomarkers for disease subset stratification and risk for organ involvement or cancer

Other names
RNPC3RNA binding region containing 3RNP1-containing protein 3
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Mechanism of action

not applicable; no known drugs act directly on RNPC3 or modulate its action

03

Biological functions

Pre-mRNA splicing (specifically U12-dependent/minor spliceosome pathway)RNA binding (binds 3'-stem-loop of U12 snRNA)Molecular bridging in U11/U12 RNP complex formationRegulation of gene expression at RNA level
04

Disease associations

Pituitary hormone deficiency, isolated or combinedScleroderma/systemic sclerosis (as an autoantigen/biomarker)Mixed connective tissue disease (systemic sclerosis skin involvement, interstitial lung disease, severe gastrointestinal disease, cancer association as biomarker)
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Safety considerations

Not directly applicable (RNPC3 is not known to be a current drug target); however, autoimmunity against RNPC3 is associated with disease states (e.g., scleroderma, interstitial lung disease)
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Biomarkers

Anti-RNPC3 autoantibodies (used as biomarkers in subsets of scleroderma and mixed connective tissue disease, associated with risk for certain organ complications and cancer)

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