... reduced Hedgehog signaling between Smo and Sufu, expanded brain size and polydactyly. Dzip1l Chibby Bromi Cilia Hedgehog Gli2 Gli3 Mouse National Institutes of Health 10.13039/100000002 R01GM114429 R01HL107493 National Natural Science Foundation of China 10.13039...

...Zhu Juan Li; Erica Nieuwenhuis; Weilun Nien; Xiaoyun Zhang; Jennifer Zhang; Vijitha Puviindran; Brandon J. Wainwright; Peter C. W. Kim; Chi-chung Hui Abnormal activation of Hedgehog (Hh) signaling leads to basal cell carcinoma (BCC) of the skin, the most common human cancer. Gli2, the major...

...) or suppressor of fused (Sufu). Genetic analysis shows that although PKA is important for production of the repressor form of Gli3, the principal function of PKA in the Shh pathway in neural development is to restrain activation of Gli2. Activation of the Hh pathway in PKA mutants depends on cilia...

...Chengbing Wang; Yong Pan; Baolin Wang Gli2 and Gli3 are primary transcriptional regulators that mediate hedgehog (Hh) signaling. Mechanisms that stabilize and destabilize Gli2 and Gli3 are essential for the proteins to promptly respond to Hh signaling or to be inactivated following the activation...

... differentiation and hypertrophic cartilage vascularization require additional mechanisms. To test the involvement of Gli2 activation in these processes, we have generated a mouse strain that expresses a constitutive Gli2 activator in a Cre-dependent manner, and have attempted to rescue the Ihh -null mouse...

... fates in part through Gli3 , but not Gli2 . These data reveal a crucial role of Wnt signaling in regulating ventral cell fates during normal vertebrate development. The genotyping of Gli1-CreER, Gli2 zfd ,Gli3 xt , conditional loss-of-function β-catenin( Ctnnb1 tm1Max ), stabilized β...

... activation,and inhibition of ERK1/2 activation blocked seminal vesicle branching morphogenesis. Partial loss of FGFR2(IIIb) was also associated with down-regulation of several branching morphogenesis regulators including Shh, Ptch1, Gli1, Gli2, Bmp4 , and Bmp7 . Together with previous studies, these data...

...)signaling correlates spatially and temporally with fissure formation, and that Gli2 is the main activator driving Shh induced proliferation of embryonic GCPs. Here, we have tested whether the level of Shh signaling regulates the extent of cerebellar foliation. By progressively lowering signaling by removing...

... differentiation,Gli3 seems to position the domain of PTHrP expression. * Author for correspondence (e-mail: [email protected] ) 26 9 2005 ©2005. 2005 Indian hedgehog (Ihh) Gli3 Gli2 Gli1 PTHrP (Pthlh) Chondrocyte Cartilage Bone The onset of hypertrophic...

... in posterior specification, the network coordinating their effects remains elusive. Here we have analyzed the function of Gli zinc-finger proteins in mesodermal A-P patterning. We find that Gli2 is sufficient to induce ventroposterior development, functioning in the FGF-brachyury regulatory loop. Gli2 directly...

.... In this study, regulatory activities associated with the Gli2 protein were investigated in relation to the Shh signaling. Although Gli2 acts as a weak transcriptional activator, it is in fact a composite of positive and negative regulatory domains. In cultured cells, truncation of the activation domain in the C...

... in providing cues to commissural axons in mice, we have analyzed Gli2 mouse mutants, which lack specifically the floor plate and immediately adjacent interneurons. We show that a Dbx1 enhancer drives tau-lacZ expression in a subpopulation of commissural axons and, using a reporter line generated from...

...) cells. These assays show that C-terminal sequences are required for positive inducing activity and cytoplasmic localization, whereas N-terminal sequences determine dominant negative function and nuclear localization. Analyses of nuclear targeted Gli1 and Gli2 proteins suggest that both activator...

... of the CNS in Gli2 mouse mutants. We have found that the floor plate throughout the midbrain, hindbrain and spinal cord does not form in Gli2 homozygotes. Despite this, motoneurons and ventral interneurons form in their normal d/v positions at 9.5 to 12.5 days postcoitum (dpc). However, cells...

...Zoë Hardcastle; Rong Mo; Chi-chung Hui; Paul T. Sharpe ABSTRACT The expression of genes involved in the Sonic Hedgehog signalling pathway, including Shh, Ptc, Smo, Gli1, Gli2 and Gli3 , were found to be expressed in temporal and spatial patterns during early murine tooth development, suggestive...

... in mutant mice lacking Gli2 function. Gli2 mutants fail to develop a floor plate yet still develop motor neurons, which occupy the ventral midline of the neural tube. Our results imply that Gli2 is required to mediate high level but not low level Shh activity and show that the development of motor neurons...