Genotype-phenotype correlations of UBA2 mutations in patients with ectrodactyly.


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 2020
Historique:
received: 31 03 2020
revised: 05 06 2020
accepted: 10 07 2020
pubmed: 8 8 2020
medline: 1 5 2021
entrez: 8 8 2020
Statut: ppublish

Résumé

Interstitial 19q13.11 deletions are associated with ectrodactyly, which has recently been linked to loss-of-function of the UBA2 gene. We report a boy with a de novo frameshift mutation in UBA2 (c.612delA (p.(Glu205Lysfs*63)), presenting with ectrodactyly of the feet associated with learning difficulties and minor physical anomalies. We review genotype-phenotype correlations in patients with chromosomal 19q13.11 microdeletions compared to those with intragenic UBA2 mutations.

Identifiants

pubmed: 32758660
pii: S1769-7212(20)30282-2
doi: 10.1016/j.ejmg.2020.104009
pii:
doi:

Substances chimiques

UBA2 protein, human 0
Ubiquitin-Activating Enzymes EC 6.2.1.45

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

104009

Informations de copyright

Copyright © 2020 Elsevier Masson SAS. All rights reserved.

Auteurs

Mio Aerden (M)

Center for Human Genetics, University Hospital Leuven and KU Leuven, Leuven, Belgium.

Marijke Bauters (M)

Center for Human Genetics, University Hospital Leuven and KU Leuven, Leuven, Belgium.

Kris Van Den Bogaert (K)

Center for Human Genetics, University Hospital Leuven and KU Leuven, Leuven, Belgium.

Joris R Vermeesch (JR)

Center for Human Genetics, University Hospital Leuven and KU Leuven, Leuven, Belgium.

Maureen Holvoet (M)

Center for Human Genetics, University Hospital Leuven and KU Leuven, Leuven, Belgium.

Frank Plasschaert (F)

Department of Physical Medicine and Orthopaedic Surgery, Ghent University, Ghent, Belgium.

Koenraad Devriendt (K)

Center for Human Genetics, University Hospital Leuven and KU Leuven, Leuven, Belgium. Electronic address: koenraad.devriendt@uzleuven.be.

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Classifications MeSH