Congenital diaphragmatic hernia as a part of Nance-Horan syndrome?
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 prese...
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Veröffentlicht in: | European journal of human genetics : EJHG 2018-03, Vol.26 (3), p.359-366 |
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Sprache: | eng |
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Zusammenfassung: | 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. |
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ISSN: | 1018-4813 1476-5438 |
DOI: | 10.1038/s41431-017-0032-z |