Cerebellar ataxia Dysmorphology Whole exome sequencing

Journal

Molecular syndromology
ISSN: 1661-8769
Titre abrégé: Mol Syndromol
Pays: Switzerland
ID NLM: 101525192

Informations de publication

Date de publication:
Jan 2019
Historique:
accepted: 29 08 2018
entrez: 26 2 2019
pubmed: 26 2 2019
medline: 26 2 2019
Statut: ppublish

Résumé

We report on a girl, born to first-cousin Lebanese parents, with severe intellectual disability, congenital hip luxation, cardiac malformation, short stature, facial dysmorphic features including microcephaly, sparse hair, bilateral epicanthal folds, ataxia, seizures, and elevated lactate and pyruvate levels in serum. Whole exome sequencing was carried out on the patient's DNA. Potentially causal homozygous variants in the

Identifiants

pubmed: 30800049
doi: 10.1159/000494465
pii: msy-0009-0319
pmc: PMC6381915
doi:

Types de publication

Journal Article

Langues

eng

Pagination

319-323

Références

Am J Hum Genet. 2001 Jan;68(1):269-74
pubmed: 11112660
Am J Hum Genet. 2008 Mar;82(3):661-72
pubmed: 18319074
Cell. 2011 Sep 30;147(1):32-43
pubmed: 21962505
J Biol Chem. 2013 Sep 6;288(36):26179-87
pubmed: 23864652
Neurogenetics. 2009 Oct;10(4):375-376
pubmed: 25488817
J Med Genet. 2015 Feb;52(2):123-7
pubmed: 25527630
Endocr Res. 2015;40(3):166-71
pubmed: 25531638
J Med Genet. 2015 Mar;52(3):163-74
pubmed: 25575550
Genet Med. 2015 May;17(5):405-24
pubmed: 25741868
Hum Genet. 2015 Jun;134(6):577-87
pubmed: 25792360
Genes (Basel). 2016 Jul 27;7(8):null
pubmed: 27472364
J Genet. 2017 Dec;96(6):1005-1014
pubmed: 29321360

Auteurs

Pratibha Nair (P)

Centre for Arab Genomic Studies, Dubai, United Arab Emirates.

Maher Lama (M)

Pediatric Department El-Rassoul Hospital, Beirut, Lebanon.

Stephany El-Hayek (S)

Centre for Arab Genomic Studies, Dubai, United Arab Emirates.

Gretta Abou Sleymane (G)

Department of Laboratory Science and Technology, American University of Science and Technology, Beirut, Lebanon.

Samantha Stora (S)

Institut Jérôme Lejeune, CRB BioJeL, Paris.

Marc Obeid (M)

Department of Laboratory Science and Technology, American University of Science and Technology, Beirut, Lebanon.

Mahmoud T Al-Ali (MT)

Centre for Arab Genomic Studies, Dubai, United Arab Emirates.

Valérie Delague (V)

Aix Marseille Univ., Inserm, MMG, U 1251, Marseille, France.

André Mégarbané (A)

Institut Jérôme Lejeune, CRB BioJeL, Paris.

Classifications MeSH