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GTR Home > Conditions/Phenotypes > Developmental and epileptic encephalopathy 115

Summary

Developmental and epileptic encephalopathy-115 (DEE115) is an autosomal recessive disorder characterized by severe developmental delay and epileptic encephalopathy, massive reduction of white matter, hypo-/aplasia of the corpus callosum, neurodevelopmental arrest, and early death (Brugger et al., 2024). For general phenotypic information and a discussion of genetic heterogeneity of DEE, see 308350. [from OMIM]

Genes See tests for all associated and related genes

  • Also known as: DEE115, Dot3, EAP30, NEDOA, VPS22, SNF8
    Summary: SNF8 subunit of ESCRT-II

Clinical features

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