ZNF526

Chr 19AR

zinc finger protein 526

Also known as: DENNED

NCBI Gene: 116115 ENSG00000167625 UniProt: Q8TF50 OMIM: 614387

ZNF526 encodes a probable transcription factor that binds DNA and zinc ions in the nucleus. Mutations cause Dentici-Novelli neurodevelopmental syndrome with autosomal recessive inheritance. The gene is highly constrained against loss-of-function variants (LOEUF 0.47), indicating that complete loss of function is likely pathogenic.

OMIM ResearchSummary from RefSeq, OMIM, UniProt

Predicted to enable DNA binding activity and zinc ion binding activity. Predicted to be located in nucleus. [provided by Alliance of Genome Resources, Jul 2025]

OMIM ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Dentici-Novelli neurodevelopmental syndromeMIM #619877

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.Curated gene-level mechanism — a prior for triage, not a per-variant call.

Active trials

Pubs (1 yr)

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P/LP submissions

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P/LP missense

0.47

LOEUF

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Mechanism