| Literature DB >> 29358614 |
Molka Kammoun1, Paul Brady1, Luc De Catte2, Jan Deprest2, Koenraad Devriendt1, Joris Robert Vermeesch3.
Abstract
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.Entities:
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Year: 2018 PMID: 29358614 PMCID: PMC5839023 DOI: 10.1038/s41431-017-0032-z
Source DB: PubMed Journal: Eur J Hum Genet ISSN: 1018-4813 Impact factor: 4.246