signaling Drosophila Hedgehog Hh pointy denticles Tabins lab identfied 3 Vertebrate genes with homology to Hh Sonic Hedgehog Shh Widespread useage in dev Particularly in NT and ID: 551162
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Slide1
Hedgehog and Notch
signaling
Slide2
Drosophila Hedgehog (Hh) pointy denticles
Tabin’s lab identfied 3 Vertebrate genes with homology to Hh:Sonic Hedgehog (Shh) Widespread useage in dev. Particularly in NT and limb developmentDesert Hedgehog (Dhh) Sertoli cells mice -/- defective spermatogenesisIndian Hedgehog (Ihh) gut and cartilage
Hedgehogs: role in vertebrate development and
some Human congenital malformation syndromesSlide3
Figure 3.25 Hedgehog signal transduction pathwaySlide4
Figure 3.26 Sonic hedgehog is shown by in situ hybridization to be expressed in the nervous system (red arrow), gut (blue arrow), and limb bud (black arrow) of a 3-day chick embryoSlide5
Model of major postulated interactions in the patterning of the somite
Signaling between NT, Nc and somite Slide6
Inherited congenital malformation syndromes
in HumansGrieg cephalopolysyndactyly
: high forehead and
increased # of digits(results from a non functional truncation
of the Gli3 protein)
Pallister Hall syndrome
lethal shortly after birth- Gli3 DBD intact
but no TAD (Dom Neg)
Ectopic activation of Hh pathway in somatic tissues
:
Eg heritable mut in Patched gene (no inhibition of Smo):
Developmental abnormalities, fused fingers, rib and facial defects
Basal cell carcinomasSlide7
Skeletal
pattern of the chick wingSlide8
Sonic
hedgehog protein is expressed in the ZPA Slide9Slide10Slide11Slide12
Head of a cyclopic lamb born of a ewe who had eaten Veratrum
californicum
early in pregnancySlide13
Figure 3.33 Mechanism of Notch activity (Part 1)Slide14
Figure 3.33 Mechanism of Notch activity (Part 2)Slide15