ZRANB1

Chr 10

zinc finger RANBP2-type containing 1

Also known as: TRABID

NCBI Gene: 54764 ENSG00000019995 UniProt: Q9UGI0

The protein is a deubiquitinating enzyme that removes specific ubiquitin linkages from target proteins and regulates Wnt signaling, autophagy, and cytoskeletal organization. Mutations cause autosomal dominant neurodevelopmental disorders with intellectual disability, developmental delay, and movement abnormalities. This gene is highly constrained against loss-of-function variants in the population, indicating that such mutations are likely pathogenic.

ResearchSummary from RefSeq, UniProt

Enables K63-linked polyubiquitin modification-dependent protein binding activity and cysteine-type deubiquitinase activity. Involved in positive regulation of Wnt signaling pathway and protein deubiquitination. Located in cytosol and nucleoplasm. Is active in cytoplasm and nucleus. [provided by Alliance of Genome Resources, Jun 2026]

ResearchGenerating clinical summary…

Clinical highlights

Interpreting a novel variant

This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.

Active trials

Pubs (1 yr)

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P/LP submissions

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P/LP missense

0.25

LOEUF· LoF intol.

LOF

Mechanism· predicted

Some data sources returned errors (1)

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