06-P033 Ablation of Dgcr8 in neural crest cells leads to cardiovascular defects reminiscent of DiGeorge syndrome

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06-P033 Ablation of Dgcr8 in neural crest cells leads to cardiovascular defects reminiscent of DiGeorge syndrome

By E16.5, the secondary lens fibre cells were abnormally arranged with poor lens suture formation. Apoptotic cells were found in the centre of the lens as shown by TUNEL assay, the cytoskeleton and cell adhesion in the lens centre were disturbed as shown in immunohistochemistry analysis. By western blotting we found that mutant c-crystallins were reduced in amount and enriched in the insoluble ...

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Loss of microRNAs in neural crest leads to cardiovascular syndromes resembling human congenital heart defects.

OBJECTIVE Congenital heart defects represent the most common human birth defects. Even though the genetic cause of these syndromes has been linked to candidate genes, the underlying molecular mechanisms are still largely unknown. Disturbance of neural crest cell (NCC) migration into the derivatives of the pharyngeal arches and pouches can account for many of the developmental defects. The goal ...

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Disruption of CXCR4 signaling in pharyngeal neural crest cells causes DiGeorge syndrome-like malformations.

DiGeorge syndrome (DGS) is a congenital disease causing cardiac outflow tract anomalies, craniofacial dysmorphogenesis, thymus hypoplasia, and mental disorders. It results from defective development of neural crest cells (NCs) that colonize the pharyngeal arches and contribute to lower jaw, neck and heart tissues. Although TBX1 has been identified as the main gene accounting for the defects obs...

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Shortened outflow tract leads to altered cardiac looping after neural crest ablation.

BACKGROUND Congenital conotruncal malformations frequently involve dextroposed aorta. The pathogenesis of dextroposed aorta is not known but is thought to be due to abnormal looping and/or wedging of the outflow tract during early heart development. We examined the stage of cardiac looping in an experimental model of dextroposed aorta to determine the embryogenesis of this conotruncal malformat...

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Genetic ablation of neural crest cell diversification.

The neural crest generates multiple cell types during embryogenesis but the mechanisms regulating neural crest cell diversification are incompletely understood. Previous studies using mutant zebrafish indicated that foxd3 and tfap2a function early and differentially in the development of neural crest sublineages. Here, we show that the simultaneous loss of foxd3 and tfap2a function in zebrafish...

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ژورنال

عنوان ژورنال: Mechanisms of Development

سال: 2009

ISSN: 0925-4773

DOI: 10.1016/j.mod.2009.06.259