Congenital diaphragmatic hernia as a part of Nance-Horan syndrome?
Eur J Hum Genet
; 26(3): 359-366, 2018 03.
Article
em En
| MEDLINE
| ID: mdl-29358614
Nance-Horan syndrome is a rare X-linked developmental disorder characterized by bilateral congenital cataract, dental anomalies, facial dysmorphism, and intellectual disability. Here, we identify a patient with Nance-Horan syndrome caused by a new nonsense NHS variant. In addition, the patient presented congenital diaphragmatic hernia. NHS gene expression in murine fetal diaphragm was demonstrated, suggesting a possible involvement of NHS in diaphragm development. Congenital diaphragmatic hernia could result from NHS loss of function in pleuroperitoneal fold or in somites-derived muscle progenitor cells leading to an impairment of their cells migration.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Fenótipo
/
Anormalidades Dentárias
/
Catarata
/
Doenças Genéticas Ligadas ao Cromossomo X
/
Hérnias Diafragmáticas Congênitas
Tipo de estudo:
Prognostic_studies
Limite:
Animals
/
Humans
/
Male
/
Newborn
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article