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This Review contains major "Shh"- related terms, short phrases and links grouped together in the form of Encyclopedia article.
- SHH is the major gene implicated in HPE and it plays a critical role in early forebrain and central nervous system development.
- SHH is the major gene implicated in holoprosencephaly (12.7% of HPE cases: 50% of overall point mutations and 38% of overall large deletions) [ 50, 51].
- Shh is the most extensively characterized vertebrate homolog, and is involved in morphogenesis of several organs including the eye, hair and lungs.
- SHH is expressed in the human embryo in the notochord, the floorplate of the neural tube, and the posterior limb buds.
- SHH was initially described as a protein secreted from two signalling centres, the notochord and the floor plate.
- Name two functions of sonic hedgehog (SHH). ------ It is produced by the notochord and it: 1.
- One can ask how Gli proteins act and what the nature of Shh signaling during CNS dorsal-ventral patterning is.
- What is not known is whether this phenotype is completely independent of Shh signaling.
- Runx2 (Cbfa1) inhibits Shh signaling in the lower but not upper molars of mouse embryos and prevents the budding of putative successional teeth.
- Shh stimulated neuronal differentiation; however, it did not increase the proportion of neurons expressing the first postmitotic motoneuron marker Islet-1.
- In some embodiments of either aspect, the activator of the SHH pathway is SHH or purmorphamine.
- S, Distribution of Shh protein demonstrated by immunostaining with anti-Shh serum (Marti et al., 1995a).
- A second possibility was that Shh actually inhibited the differentiation of Isl-1 motor neurons, as suggested from in vitro studies (Kalyani et al., 1998).
- The underlying notochord produces the well-known transcription factor, Sonic hedgehog ("Shh") .
- In the absence of Shh, BMP administration induces lateral plate gene expression in cultured psm.
- PTC, a vertebrate homolog of this Drosophila gene, is genetically downstream of Sonic hedgehog (Shh) in the limb bud.
- Sonic hedgehog (Shh) up-regulates Gli1 transcription but down-regulates Gli3 expression (Marigo et al.
- Therefore, the differentiation of OPCs was strictly dependent upon SHH.
- These Olig2 progenitors no longer produced motor neurons, but instead generated OPCs after eight additional weeks of SHH treatment.
- Tiarin directly dorsalizes neural tissues in the absence of mesodermal tissues and antagonizes the ventralizing activity of Sonic hedghog (Shh).
- The exact pattern of marker gene expression and eventual cell fate is thought to depend on the concentration of Shh (Ericson et al.
- Coexpression of c-Ski with Shh and Gli3 significantly lowered the level of induction of Gli1 mRNA by about 3.8-fold.
- Like Shh mutants and Gli2 mutants (Chiang et al.
- We found that the abrogation of Ptch expression exhibited by Shh mutants is indeed partially alleviated by loss of Tectonic function (Fig.
- They could subsequently enhance the repressive activity of PTCH on the SHH pathway and so decrease SHH signaling.
- At day 35, OLIG2 and NKX2.2 co-express only in cultures with SHH or purmorphamine.
- In contrast to Gli1 and Gli2, there is little doubt that Gli3 acts as a repressor of Hh targets as well as of Shh itself (Masuya et al.
- However, Shh signaling is not essential for differentiation of ameloblasts or odontoblasts.
- However, in preliminary electroporation experiments using activators of the Wnt pathway we never observed downregulation of Shh in the ZLI (data not shown).
- Lanosterol synthase catalyzes a key step in the biosynthesis of cholesterol, essential in the maturation of the SHH protein.
- In addition, there is evidence that Shh signaling may specify oligodendrocyte precursors (Poncet et al., 1996; Pringle et al., 1996).
- In addition to identifying expected Gli-target sites, the data predicted a number of unreported direct targets of Shh action.
- In addition, Gli3 is itself a target of transcriptional repression by Shh (Marigo et al.
- Shh cannot act alone to induce a floor plate.
- I, L, Expression of HES-1 was upregulated in Shh -Tg (arrow) in cells lining the VZ. J, M, Pax-6 was maintained in a similar pattern of expression (arrow).
- First, we used Shh as a molecular marker to establish a precise temporal sequence for rugae appearance in mouse.
- SIL is a cytosolic protein required in mouse for left-right axis development and Shh signaling (Izraeli et al., 2001; Izraeli et al., 1999).
- These results point out that not all the midbrain neuronal populations are dependent on Shh for their maintenance, as previously thought.
- I, J, Maintenance and expansion of roofplate cells indicated by expression of Wnt-3a in Shh -Tg tissue.
- In addition, some of the factors with pro-neurogenic effects (such as NT-3 and Shh) can be secreted by GABAergic interneurons .
- That is, we cannot with certainty establish a cause-effect relationship between the absence of Shh expression and the formation of the rosettes.
- The floor plate then produces its own SHH which results in motor neurons developing on either side.
- These results suggest that the neural tube angiogenesis is dependent on Shh signaling, and mediated, at least in part, by the Ang-1 positive motor neurons.
- These results confirmed that the oligodendrocyte lineage was induced in the hyperplastic tissue surrounding the VZ in Shh -Tg mice.
- PATCHED-1 (PTCH) is the receptor for SHH, and normally acts to repress SHH signaling.
- Growing evidence suggests that the disorder may result from mutations in genes of the Sonic hedgehog (Shh) signalling pathway.
- These patterning defects are qualitatively similar to those caused by mutations in Shh or Gli2 (Chiang et al.
- Q, R, Wild-type and Shh -Tg cervical spinal cord tissues showing expression of Shh.
- SHH remains important in the adult.
- Moreover, Shh did induce Islet-1 expression in neural tube explants, suggesting that it acts in combination with neural tube factors to induce motoneurons.
- Shh mediates its effects by binding to its receptor, Patched (Ptch).
- This is a dynamic process in which increasing concentrations and durations of exposure to SHH generate neurons with successively more ventral identities.
- In the present study we performed mutational analysis of the entire coding region of the SHH gene in 37 unrelated individuals with the HPE spectrum.
- The sonic hedgehog (Shh) gene has been identified in defects that cause hydrocephalus secondary to holoprosencephaly.
- In vitro, the inductive properties of the notochord are mimicked by Shh (Fan et al.
- The hypothesised mechanism suggests that Shh binds patched, relieving patched inhibition of smoothened, leading to activation of glia transcription factors.
- The determination of diverse cell fates by Shh signaling occurs by regulating the combination of Gli genes expressed in a cell.
- In addition to its role in ventral neural tube patterning, SHH participates in the control of progenitor cell number in developing dorsal brain.
- These results suggest that Shh may play a role in the normal inductive interactions that pattern the ventral CNS.
- Incardona JP, Roelink H: The role of cholesterol in Shh signaling and teratogen-induced holoprosencephaly.
- Here, for the first time, we analyze in detail the development of the different mesencephalic basal nuclei in the absence of Shh.
- Encyclopedia of Keywords > Information > Structure > Neural Tube
* Bmp Signaling
* Cell Types
* Concentration-Dependent Manner
* Ectopic Expression
* Floor Plate
* Motor Neurons
* Neural Tube
* Shh -Tg Mice
* Shh Signaling
* Signaling Molecule
* Signal Transduction
* Sonic Hedgehog
* Spinal Cord
* Ventral Neural Tube
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