The relationship between sonic Hedgehog signaling, cilia, and neural tube defects
Autor: | Jennifer N. Murdoch, Andrew J. Copp |
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Rok vydání: | 2010 |
Předmět: |
Embryology
medicine.medical_specialty animal structures Neural tube patterning Exencephaly Article Mice Holoprosencephaly Pregnancy Intraflagellar transport Internal medicine medicine Animals Humans Hedgehog Proteins Cilia Neural Tube Defects Sonic hedgehog Floor plate biology Neural tube General Medicine medicine.disease Hedgehog signaling pathway Cell biology Endocrinology medicine.anatomical_structure embryonic structures Pediatrics Perinatology and Child Health biology.protein Female Signal Transduction Developmental Biology |
Zdroj: | Birth Defects Research Part A: Clinical and Molecular Teratology. 88:633-652 |
ISSN: | 1542-0752 |
DOI: | 10.1002/bdra.20686 |
Popis: | The Hedgehog signaling pathway is essential for many aspects of normal embryonic development, including formation and patterning of the neural tube. Absence of the sonic hedgehog (shh) ligand is associated with the midline defect holoprosencephaly, whereas increased Shh signaling is associated with exencephaly and spina bifida. To complicate this apparently simple relationship, mutation of proteins required for function of cilia often leads to impaired Shh signaling and to disruption of neural tube closure. In this article, we review the literature on Shh pathway mutants and discuss the relationship between Shh signaling, cilia, and neural tube defects. |
Databáze: | OpenAIRE |
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