Biallelic HERC1 mutations in a syndromic form of overgrowth and intellectual disability

We report two Colombian siblings affected by overgrowth, intellectual disability and facial dysmorphism. Exome (via NGS) and Sanger sequencing revealed that biallelic sequence variants in a novel gene (HERC1) might be related to the disease pathogenesis. These results provide useful data for future...

ver descrição completa

Detalhes bibliográficos
Autores: Ortega?Recalde, O., Beltrán, O.I., Gálvez, J.M., Palma?Montero, A., Restrepo Fernández, Carlos Martín, Mateus, H.E., Laissue, P.
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2015
País:Colombia
Recursos:Universidad del Rosario
Repositorio:Repositorio EdocUR - U. Rosario
Idioma:inglés
OAI Identifier:oai:repository.urosario.edu.co:10336/23680
Acesso em linha:https://doi.org/10.1111/cge.12634
https://repository.urosario.edu.co/handle/10336/23680
Access Level:acceso abierto
Palavra-chave:Allele
Article
Case report
Clinical feature
Colombian
Exome
Face dysmorphia
Female
Gene
Genotype phenotype correlation
Herc 1 gene
Human
Intellectual impairment
Male
Molecular diagnosis
Newborn
Pathogenesis
Priority journal
Sequence analysis
Sibling
Dna mutational analysis
Genetic association study
Genetics
Growth disorder
Human genome
Mutation
Pathology
Syndrome
Guanine nucleotide exchange factor
Genetic association studies
Growth disorders
Guanine nucleotide exchange factors
Humans
Intellectual disability
Exome sequencing
Herc1 mutations
Overgrowth
human
Herc1 protein
Genome
Descrição
Resumo:We report two Colombian siblings affected by overgrowth, intellectual disability and facial dysmorphism. Exome (via NGS) and Sanger sequencing revealed that biallelic sequence variants in a novel gene (HERC1) might be related to the disease pathogenesis. These results provide useful data for future genotype-phenotype correlations and for a molecular diagnosis of overgrowth. © 2015 John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.