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PURA-Related Developmental and Epileptic...
Journal article

PURA-Related Developmental and Epileptic Encephalopathy

Abstract

BACKGROUND AND OBJECTIVES: Purine-rich element-binding protein A (PURA) gene encodes Pur-α, a conserved protein essential for normal postnatal brain development. Recently, a PURA syndrome characterized by intellectual disability, hypotonia, epilepsy, and dysmorphic features was suggested. The aim of this study was to define and expand the phenotypic spectrum of PURA syndrome by collecting data, including EEG, from a large cohort of affected …

Authors

Johannesen KM; Gardella E; Gjerulfsen CE; Bayat A; Rouhl RPW; Reijnders M; Whalen S; Keren B; Buratti J; Courtin T

Journal

Neurology Genetics, Vol. 7, No. 6,

Publisher

Wolters Kluwer

Publication Date

December 2021

DOI

10.1212/nxg.0000000000000613

ISSN

2376-7839