The relationship between sonic Hedgehog signaling, cilia, and neural tube defects

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...

Full description

Saved in:
Bibliographic Details
Published inBirth defects research. A Clinical and molecular teratology Vol. 88; no. 8; p. 633
Main Authors Murdoch, Jennifer N, Copp, Andrew J
Format Journal Article
LanguageEnglish
Published United States 01.08.2010
Subjects
Animals
Cilia - genetics
Cilia - metabolism
Female
Hedgehog Proteins - genetics
Hedgehog Proteins - metabolism
Holoprosencephaly - genetics
Holoprosencephaly - metabolism
Humans
Mice
Neural Tube Defects - genetics
Neural Tube Defects - metabolism
Pregnancy
Signal Transduction
Online AccessGet more information

Cover

More Information
Summary: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.
ISSN:1542-0760
DOI:10.1002/bdra.20686