Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 1 de 1
Filtrar
Mais filtros

Base de dados
Ano de publicação
Tipo de documento
Intervalo de ano de publicação
1.
Am J Med Genet A ; 161A(7): 1594-8, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23704076

RESUMO

We report on three males with de novo overlapping 7.5, 9.8, and 10 Mb duplication of chromosome 20q11.2. Together with another patient previously published in the literature with overlapping 20q11 microduplication, we show that such patients display common clinical features including metopic ridging/trigonocephaly, developmental delay, epicanthal folds, and short hands. The duplication comprised the ASXL1 gene, in which de novo heterozygous nonsense or truncating mutations have recently been reported in patients with Borhing-Opitz syndrome. Because of craniofacial features in common with Borhing-Opitz syndrome, in particular metopic ridging/trigonocephaly, we suggest that duplication of ASXL1 contributes to the phenotype. These observations suggest a novel microduplication syndrome, and reporting of additional patients with molecular characterization will allow more detailed genotype-phenotype correlations.


Assuntos
Craniossinostoses/genética , Proteínas Repressoras/genética , Trissomia/genética , Criança , Pré-Escolar , Cromossomos Humanos Par 20/genética , Deficiências do Desenvolvimento/genética , Feminino , Deformidades Congênitas da Mão/genética , Heterozigoto , Humanos , Lactente , Deficiência Intelectual/genética , Masculino , Mosaicismo , Mutação , Gravidez , Síndrome
SELEÇÃO DE REFERÊNCIAS
Detalhe da pesquisa