UBTF Mutation Causes Complex Phenotype of Neurodegeneration and Severe Epilepsy in Childhood.


Journal

Neuropediatrics
ISSN: 1439-1899
Titre abrégé: Neuropediatrics
Pays: Germany
ID NLM: 8101187

Informations de publication

Date de publication:
02 2019
Historique:
pubmed: 6 12 2018
medline: 31 12 2019
entrez: 6 12 2018
Statut: ppublish

Résumé

Neurodegenerative diseases of childhood present with progressive decline in cognitive, social, and motor function and are frequently associated with seizures in different stages of the disease. Here we report a patient with severe progressive neurodegeneration with drug-resistant epilepsy of unknown etiology from the age of 2 years. Using whole exome sequencing, we found heterozygous missense de novo variant c.628G > A (p.Glu210Lys) in the Our report expands the complex phenotype of neurodegeneration associated with the c.628G > A variant in the

Identifiants

pubmed: 30517966
doi: 10.1055/s-0038-1676288
doi:

Substances chimiques

Pol1 Transcription Initiation Complex Proteins 0
transcription factor UBF 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

57-60

Informations de copyright

Georg Thieme Verlag KG Stuttgart · New York.

Déclaration de conflit d'intérêts

None of the authors has any conflicts of interest to disclose.

Auteurs

Lucie Sedláčková (L)

DNA Laboratory, Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Petra Laššuthová (P)

DNA Laboratory, Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Katalin Štěrbová (K)

Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Jana Haberlová (J)

Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Emílie Vyhnálková (E)

Department of Biology and Medical Genetics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Jana Neupauerová (J)

DNA Laboratory, Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

David Staněk (D)

DNA Laboratory, Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Marie Šedivá (M)

Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Pavel Kršek (P)

Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Pavel Seeman (P)

DNA Laboratory, Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

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Classifications MeSH