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HUWE1 variants cause dominant X-linked intellectual disability: a clinical study of 21 patients

Abstract:

Whole-gene duplications and missense variants in the HUWE1 gene (NM_031407.6) have been reported in association with intellectual disability (ID). Increased gene dosage has been observed in males with non-syndromic mild to moderate ID with speech delay. Missense variants reported previously appear to be associated with severe ID in males and mild or no ID in obligate carrier females. Here, we report the largest cohort of patients with HUWE1 variants, consisting of 14 females and 7 males, with...

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Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1038/s41431-017-0038-6

Authors


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Institution:
University of Oxford
Oxford college:
Pembroke College
Role:
Author
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Funding agency for:
Wilkie, A
Grant:
Senior Investigator Award (102731
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Grant:
PI14/00350 /Acción Estratégica en Salud 2013-2016
FEDER -Fondo Europeo de Desarrollo Regional
Institut de Recherche Scientifique en Pathologie et Génétique More from this funder
Publisher:
Springer Nature Publisher's website
Journal:
European Journal of Human Genetics Journal website
Volume:
26
Pages:
64–74
Publication date:
2017-11-27
Acceptance date:
2017-09-10
DOI:
ISSN:
1476-5438
Pubs id:
pubs:729237
UUID:
uuid:6ec021fe-43a3-4eb9-8359-199206c6a7b9
Local pid:
pubs:729237
Source identifiers:
729237
Deposit date:
2017-09-18

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