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Intellectual disability v9.389 PGBD5 Karen Stals gene: PGBD5 was added
gene: PGBD5 was added to Intellectual disability. Sources: Literature
Mode of inheritance for gene: PGBD5 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: PGBD5 were set to PMID: 41533792
Phenotypes for gene: PGBD5 were set to Seizures; global developmental delay; spasticity; hypotonia
Penetrance for gene: PGBD5 were set to Complete
Review for gene: PGBD5 was set to GREEN
gene: PGBD5 was marked as current diagnostic
Added comment: Zapater et al. 2026 PMID:41533792 report 5 families with homozygous loss of function variants in PGBD5 co-segregating in two affected siblings in each family (variants identified via whole exome sequencing). Authors show that PGBD5 contributes to normal brain development in mice and humans.
Sources: Literature