A previously identified missense mutation in STYXL1 is likely benign.


Journal

European journal of medical genetics
ISSN: 1878-0849
Titre abrégé: Eur J Med Genet
Pays: Netherlands
ID NLM: 101247089

Informations de publication

Date de publication:
Nov 2019
Historique:
received: 12 09 2018
revised: 15 11 2018
accepted: 22 11 2018
pubmed: 26 11 2018
medline: 7 2 2020
entrez: 26 11 2018
Statut: ppublish

Résumé

Based on a homozygous missense variant p.Pro311Ala found in three siblings of a consanguineous family, mutations in the STYXL1 gene were suggested to cause moderate intellectual disability, epilepsy and complex behavioural abnormalities. We have detected this variant via whole exome sequencing in a homozygous state in two families. Segregation analyses in our families and thorough validation in international genetic databases provides evidence that this variant is most likely benign. This is important information for genetic counselling. The role of STYXL1 variants in human disease needs to be established.

Identifiants

pubmed: 30472486
pii: S1769-7212(18)30680-3
doi: 10.1016/j.ejmg.2018.11.016
pii:
doi:

Substances chimiques

Apoptosis Regulatory Proteins 0
STYXL1 protein, human 0

Types de publication

Letter

Langues

eng

Sous-ensembles de citation

IM

Pagination

103582

Informations de copyright

Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Auteurs

Holger Hengel (H)

Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany; German Center of Neurodegenerative Diseases (DZNE), Tübingen, Germany.

Yvonne Schelling (Y)

Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.

Reinhard Keimer (R)

Caritas Baby Hospital, Bethlehem, Palestine.

Werner Deigendesch (W)

Caritas Baby Hospital, Bethlehem, Palestine.

Peter Bauer (P)

Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.

Ludger Schöls (L)

Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany; German Center of Neurodegenerative Diseases (DZNE), Tübingen, Germany. Electronic address: ludger.schoels@uni-tuebingen.de.

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Classifications MeSH