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Congenital diaphragmatic hernia as a part of Nance-Horan syndrome?
Kammoun, Molka; Brady, Paul; De Catte, Luc; Deprest, Jan; Devriendt, Koenraad; Vermeesch, Joris Robert.
Afiliação
  • Kammoun M; Department of Human Genetics, KU Leuven, O&N I Herestraat 49, box 602, 3000, Leuven, Belgium.
  • Brady P; Department of Human Genetics, KU Leuven, O&N I Herestraat 49, box 602, 3000, Leuven, Belgium.
  • De Catte L; Department Obstetrics and Gynecology, University Hospital Leuven, Leuven, Belgium.
  • Deprest J; Department Obstetrics and Gynecology, University Hospital Leuven, Leuven, Belgium.
  • Devriendt K; Department of Human Genetics, KU Leuven, O&N I Herestraat 49, box 602, 3000, Leuven, Belgium.
  • Vermeesch JR; Department of Human Genetics, KU Leuven, O&N I Herestraat 49, box 602, 3000, Leuven, Belgium. joris.vermeesch@uzleuven.be.
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.
Assuntos

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

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