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Loss-of-function mutations in UDP-Glucose 6-Dehydrogenase cause recessive developmental epileptic encephalopathy

Authors :
Holger Hengel
Célia Bosso-Lefèvre
George Grady
Emmanuelle Szenker-Ravi
Hankun Li
Sarah Pierce
Élise Lebigot
Thong-Teck Tan
Michelle Y. Eio
Gunaseelan Narayanan
Kagistia Hana Utami
Monica Yau
Nader Handal
Werner Deigendesch
Reinhard Keimer
Hiyam M. Marzouqa
Meral Gunay-Aygun
Michael J. Muriello
Helene Verhelst
Sarah Weckhuysen
Sonal Mahida
Sakkubai Naidu
Terrence G. Thomas
Jiin Ying Lim
Ee Shien Tan
Damien Haye
Michèl A. A. P. Willemsen
Renske Oegema
Wendy G. Mitchell
Tyler Mark Pierson
Marisa V. Andrews
Marcia C. Willing
Lance H. Rodan
Tahsin Stefan Barakat
Marjon van Slegtenhorst
Ralitza H. Gavrilova
Diego Martinelli
Tal Gilboa
Abdullah M. Tamim
Mais O. Hashem
Moeenaldeen D. AlSayed
Maha M. Abdulrahim
Mohammed Al-Owain
Ali Awaji
Adel A. H. Mahmoud
Eissa A. Faqeih
Ali Al Asmari
Sulwan M. Algain
Lamyaa A. Jad
Hesham M. Aldhalaan
Ingo Helbig
David A. Koolen
Angelika Riess
Ingeborg Kraegeloh-Mann
Peter Bauer
Suleyman Gulsuner
Hannah Stamberger
Alvin Yu Jin Ng
Sha Tang
Sumanty Tohari
Boris Keren
Laura E. Schultz-Rogers
Eric W. Klee
Sabina Barresi
Marco Tartaglia
Hagar Mor-Shaked
Sateesh Maddirevula
Amber Begtrup
Aida Telegrafi
Rolph Pfundt
Rebecca Schüle
Brian Ciruna
Carine Bonnard
Mahmoud A. Pouladi
James C. Stewart
Adam Claridge-Chang
Dirk J. Lefeber
Fowzan S. Alkuraya
Ajay S. Mathuru
Byrappa Venkatesh
Joseph J. Barycki
Melanie A. Simpson
Saumya S. Jamuar
Ludger Schöls
Bruno Reversade
Source :
Nature Communications, Vol 11, Iss 1, Pp 1-16 (2020)
Publication Year :
2020
Publisher :
Nature Portfolio, 2020.

Abstract

UDP-glucuronic acid is a component of the extracellular matrix. Here, the authors report biallelic variants in the gene encoding UDP-Glucose 6-Dehydrogenase (UGDH) in individuals affected by developmental epileptic encephalopathies that impair UGDH stability, oligomerization, or enzymatic activity in vitro.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
11
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.669f0574667343e2aba272d086c020c6
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-020-14360-7