Deconstructing the hedgehog pathway in development and disease.
The Hedgehog (Hh) family of secreted signaling proteins is a master regulator of cell fate determination in metazoans, contributing to both pattern formation during embryonic development and postembryonic tissue homeostasis. In a universally used mode of action, graded distribution of Hh protein induces differential cell fate in a dose-dependent manner in cells that receive Hh. Though much of this pathway has been elucidated from genetically based studies in model organisms, such as Drosophila and mice, the importance of Hh-mediated signaling in humans is clearly evident from malformations and a broad range of cancers that arise when the pathway is corrupted.
Jacob
Leni
L
Department of Cell Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
Lum
Lawrence
L
D000818:Animals; D002490:Central Nervous System / Q000196:embryology; D002923:Cilia / Q000502:physiology; D029721:Drosophila Proteins / Q000378:metabolism; D053823:Hedgehog Proteins / Q000378:metabolism*; D006801:Humans; D008954:Models, Biological; D009369:Neoplasms / Q000378:metabolism; D000072079:Patched Receptors; D011956:Receptors, Cell Surface / Q000378:metabolism; D043562:Receptors, G-Protein-Coupled / Q000378:metabolism; D015398:Signal Transduction
D029721:Drosophila Proteins; D053823:Hedgehog Proteins; D000072079:Patched Receptors; D011956:Receptors, Cell Surface; D043562:Receptors, G-Protein-Coupled; C077245:hh protein, Drosophila