RNU4-2

Chr 12AD

RNA, U4 small nuclear 2

Also known as: NEDHAFA, RENU, RNU4-1B, RNU4B1, RNU4C, RP102, U4A, U4b

This gene encodes U4 small nuclear RNA, a component of the spliceosome that binds U6 snRNA and is essential for pre-mRNA splicing through formation of the U4/U6 x U5 tri-snRNP complex. Mutations cause ReNU syndrome with autosomal dominant inheritance. The syndrome involves defects in RNA splicing that affect multiple organ systems during development.

OMIMResearchSummary from RefSeq, OMIM
LOFmechanismAD2 OMIM phenotypes
Clinical SummaryRNU4-2
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Gene-Disease Validity (ClinGen)
neurodevelopmental disorder with hypotonia, brain anomalies, distinctive facies, and absent language · ADStrong

Strong evidence — appropriate for clinical testing

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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RNU4-2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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