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Using Primary Neurosphere Cultures to Study Primary Cilia

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Published on: April 14, 2017

Multiple essential roles for primary cilia in heart development.

Marc August Willaredt¹, Karin Gorgas, Humphrey A R Gardner

¹Interdisciplinary Center for Neurosciences, University of Heidelberg, Heidelberg, 69120, Germany. Kerry.Tucker@urz.uni-hd.de.

|January 29, 2013

Summary

This summary is machine-generated.

Investigating primary cilia in heart development revealed that defects in intraflagellar transport protein 88 (Ift88) cause congenital heart defects by disrupting Sonic hedgehog and BMP signaling pathways.

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Simple Detection of Primary Cilia by Immunofluorescence

Simple Detection of Primary Cilia by Immunofluorescence

Published on: May 15, 2020

Area of Science:

Developmental Biology
Cell Biology

Background:

Primary cilia are essential microtubule-based structures involved in organ development.
Congenital heart defects (CHDs) are common and arise from various developmental disruptions.

Purpose of the Study:

To investigate the role of primary cilia in heart development using a mouse model with defects in intraflagellar transport protein 88 (Ift88).
To identify the molecular mechanisms underlying CHDs associated with primary cilia dysfunction.

Main Methods:

Generated and analyzed serial sections of mutant (cobblestone) and wild-type embryonic hearts.
Utilized 3D reconstructions, immunofluorescence confocal microscopy, and transmission electron microscopy.
Examined gene expression patterns using in situ hybridization.

Main Results:

Mutant embryos exhibited severe CHDs, including septal defects and outflow tract abnormalities.
Loss of primary cilia in mutant hearts led to disrupted Sonic hedgehog signaling and reduced Islet 1 expression.
Impaired BMP signaling (BMP2/4) in endocardial cushions contributed to septal and outflow tract defects.

Conclusions:

Primary cilia are critical for normal heart development, mediating Sonic hedgehog and BMP signaling pathways.
Dysfunction in cilia-related genes, such as Ift88, may underlie common CHDs like atrioventricular septal defects.