Ptch1

Summary

Gene Symbol: Ptch1
Description: patched 1
Alias: A230106A15Rik, Ptc, Ptc1, Ptch, mes, wig, protein patched homolog 1, Ptch1 protein +12b, mesenchymal dysplasia, patched homolog 1
Species: mouse
Products:     Ptch1

Top Publications

  1. Wang C, Pan Y, Wang B. Suppressor of fused and Spop regulate the stability, processing and function of Gli2 and Gli3 full-length activators but not their repressors. Development. 2010;137:2001-9 pubmed publisher
    ..Our study provides a new insight into the regulation of Gli2 and Gli3 stability and processing by Sufu and Spop, and reveals the unexpected Sufu-independent Gli3 repressor function...
  2. Dave R, Ellis T, Toumpas M, Robson J, Julian E, Adolphe C, et al. Sonic hedgehog and notch signaling can cooperate to regulate neurogenic divisions of neocortical progenitors. PLoS ONE. 2011;6:e14680 pubmed publisher
    ..Hence, our results demonstrate that correct corticogenesis is an outcome of the interplay between the Hh and Notch signaling pathways. ..
  3. Seifert A, Bouldin C, Choi K, Harfe B, Cohn M. Multiphasic and tissue-specific roles of sonic hedgehog in cloacal septation and external genitalia development. Development. 2009;136:3949-57 pubmed publisher
  4. Jokela H, Rantakari P, Lamminen T, Strauss L, Ola R, Mutka A, et al. Hydroxysteroid (17beta) dehydrogenase 7 activity is essential for fetal de novo cholesterol synthesis and for neuroectodermal survival and cardiovascular differentiation in early mouse embryos. Endocrinology. 2010;151:1884-92 pubmed publisher
    ..The septum of the atrium was also defected in the knockout mice. ..
  5. Atwood S, Li M, Lee A, Tang J, Oro A. GLI activation by atypical protein kinase C ?/? regulates the growth of basal cell carcinomas. Nature. 2013;494:484-8 pubmed publisher
    ..These results demonstrate that aPKC-?/? is critical for HH-dependent processes and implicates aPKC-?/? as a new, tumour-selective therapeutic target for the treatment of SMO-inhibitor-resistant cancers. ..
  6. Ribes V, Balaskas N, Sasai N, Cruz C, Dessaud E, Cayuso J, et al. Distinct Sonic Hedgehog signaling dynamics specify floor plate and ventral neuronal progenitors in the vertebrate neural tube. Genes Dev. 2010;24:1186-200 pubmed publisher
    ..In addition, we provide evidence supporting a common scheme for FP specification by Shh signaling that reconciles mechanisms of FP development in teleosts and amniotes. ..
  7. Zhang W, Hong M, Bae G, Kang J, Krauss R. Boc modifies the holoprosencephaly spectrum of Cdo mutant mice. Dis Model Mech. 2011;4:368-80 pubmed publisher
  8. Swartling F, Grimmer M, Hackett C, Northcott P, Fan Q, Goldenberg D, et al. Pleiotropic role for MYCN in medulloblastoma. Genes Dev. 2010;24:1059-72 pubmed publisher
    ..Targeted expression of MYCN thus contributes to initiation, progression, and maintenance of MB, suggesting a central role for MYCN in pathogenesis. ..
  9. Wells K, Mou C, Headon D, Tucker A. Defects and rescue of the minor salivary glands in Eda pathway mutants. Dev Biol. 2011;349:137-46 pubmed publisher
    ..Supplementation with Fgf8 or Shh, previously reported targets of Eda signalling, leads to induction of gland like structures in a few cases, but these fail to develop into minor SGs. ..
  10. Ashe A, Butterfield N, Town L, Courtney A, Cooper A, Ferguson C, et al. Mutations in mouse Ift144 model the craniofacial, limb and rib defects in skeletal ciliopathies. Hum Mol Genet. 2012;21:1808-23 pubmed publisher
    ..This allelic series of mouse mutants provides a unique opportunity to uncover the underlying mechanistic basis of this intriguing subset of ciliopathies. ..

Detail Information

Publications76

  1. Wang C, Pan Y, Wang B. Suppressor of fused and Spop regulate the stability, processing and function of Gli2 and Gli3 full-length activators but not their repressors. Development. 2010;137:2001-9 pubmed publisher
    ..Our study provides a new insight into the regulation of Gli2 and Gli3 stability and processing by Sufu and Spop, and reveals the unexpected Sufu-independent Gli3 repressor function...
  2. Dave R, Ellis T, Toumpas M, Robson J, Julian E, Adolphe C, et al. Sonic hedgehog and notch signaling can cooperate to regulate neurogenic divisions of neocortical progenitors. PLoS ONE. 2011;6:e14680 pubmed publisher
    ..Hence, our results demonstrate that correct corticogenesis is an outcome of the interplay between the Hh and Notch signaling pathways. ..
  3. Seifert A, Bouldin C, Choi K, Harfe B, Cohn M. Multiphasic and tissue-specific roles of sonic hedgehog in cloacal septation and external genitalia development. Development. 2009;136:3949-57 pubmed publisher
  4. Jokela H, Rantakari P, Lamminen T, Strauss L, Ola R, Mutka A, et al. Hydroxysteroid (17beta) dehydrogenase 7 activity is essential for fetal de novo cholesterol synthesis and for neuroectodermal survival and cardiovascular differentiation in early mouse embryos. Endocrinology. 2010;151:1884-92 pubmed publisher
    ..The septum of the atrium was also defected in the knockout mice. ..
  5. Atwood S, Li M, Lee A, Tang J, Oro A. GLI activation by atypical protein kinase C ?/? regulates the growth of basal cell carcinomas. Nature. 2013;494:484-8 pubmed publisher
    ..These results demonstrate that aPKC-?/? is critical for HH-dependent processes and implicates aPKC-?/? as a new, tumour-selective therapeutic target for the treatment of SMO-inhibitor-resistant cancers. ..
  6. Ribes V, Balaskas N, Sasai N, Cruz C, Dessaud E, Cayuso J, et al. Distinct Sonic Hedgehog signaling dynamics specify floor plate and ventral neuronal progenitors in the vertebrate neural tube. Genes Dev. 2010;24:1186-200 pubmed publisher
    ..In addition, we provide evidence supporting a common scheme for FP specification by Shh signaling that reconciles mechanisms of FP development in teleosts and amniotes. ..
  7. Zhang W, Hong M, Bae G, Kang J, Krauss R. Boc modifies the holoprosencephaly spectrum of Cdo mutant mice. Dis Model Mech. 2011;4:368-80 pubmed publisher
  8. Swartling F, Grimmer M, Hackett C, Northcott P, Fan Q, Goldenberg D, et al. Pleiotropic role for MYCN in medulloblastoma. Genes Dev. 2010;24:1059-72 pubmed publisher
    ..Targeted expression of MYCN thus contributes to initiation, progression, and maintenance of MB, suggesting a central role for MYCN in pathogenesis. ..
  9. Wells K, Mou C, Headon D, Tucker A. Defects and rescue of the minor salivary glands in Eda pathway mutants. Dev Biol. 2011;349:137-46 pubmed publisher
    ..Supplementation with Fgf8 or Shh, previously reported targets of Eda signalling, leads to induction of gland like structures in a few cases, but these fail to develop into minor SGs. ..
  10. Ashe A, Butterfield N, Town L, Courtney A, Cooper A, Ferguson C, et al. Mutations in mouse Ift144 model the craniofacial, limb and rib defects in skeletal ciliopathies. Hum Mol Genet. 2012;21:1808-23 pubmed publisher
    ..This allelic series of mouse mutants provides a unique opportunity to uncover the underlying mechanistic basis of this intriguing subset of ciliopathies. ..
  11. Mukhopadhyay S, Wen X, Ratti N, Loktev A, Rangell L, Scales S, et al. The ciliary G-protein-coupled receptor Gpr161 negatively regulates the Sonic hedgehog pathway via cAMP signaling. Cell. 2013;152:210-23 pubmed publisher
    ..Conversely, Shh signaling directs Gpr161 to be internalized from cilia, preventing its activity. Thus, Gpr161 defines a morphogenetic pathway coupling protein kinase A activation to Shh signaling during neural tube development. ..
  12. Zeng H, Hoover A, Liu A. PCP effector gene Inturned is an important regulator of cilia formation and embryonic development in mammals. Dev Biol. 2010;339:418-28 pubmed publisher
    ..We found that in Intu mutant embryos the expression of Gli1 and Ptch1, two direct transcriptional targets of Hh signaling, is down-regulated, and the proteolytic processing of Gli3 is ..
  13. Balczerski B, Zakaria S, Tucker A, Borycki A, Koyama E, Pacifici M, et al. Distinct spatiotemporal roles of hedgehog signalling during chick and mouse cranial base and axial skeleton development. Dev Biol. 2012;371:203-14 pubmed publisher
  14. Hong M, Krauss R. Cdon mutation and fetal ethanol exposure synergize to produce midline signaling defects and holoprosencephaly spectrum disorders in mice. PLoS Genet. 2012;8:e1002999 pubmed publisher
    ..Furthermore, gene-environment interactions are likely to be important in the multifactorial etiology of HPE...
  15. Lin C, Fisher A, Yin Y, Maruyama T, Veith G, Dhandha M, et al. The inductive role of Wnt-?-Catenin signaling in the formation of oral apparatus. Dev Biol. 2011;356:40-50 pubmed publisher
    ..We provide genetic evidence that disruption of either signaling pathway results in severe microglossia. Altogether, we demonstrate a dynamic role for Wnt-?-Catenin signaling in the development of the oral apparatus...
  16. Dennis J, Kurosaka H, Iulianella A, Pace J, Thomas N, Beckham S, et al. Mutations in Hedgehog acyltransferase (Hhat) perturb Hedgehog signaling, resulting in severe acrania-holoprosencephaly-agnathia craniofacial defects. PLoS Genet. 2012;8:e1002927 pubmed publisher
    ..We show through downregulation of the Hh receptor Ptch1 that loss of Hhat perturbs long-range Hh signaling, which in turn disrupts Fgf, Bmp and Erk signaling...
  17. Bruce S, Butterfield N, Metzis V, Town L, McGlinn E, Wicking C. Inactivation of Patched1 in the mouse limb has novel inhibitory effects on the chondrogenic program. J Biol Chem. 2010;285:27967-81 pubmed publisher
    ..We have conditionally inactivated the hedgehog receptor Ptc1 in undifferentiated limb mesenchyme of the mouse limb using Prx1-Cre, thus inducing constitutively active ligand-..
  18. Nitzki F, Zibat A, König S, Wijgerde M, Rosenberger A, Brembeck F, et al. Tumor stroma-derived Wnt5a induces differentiation of basal cell carcinoma of Ptch-mutant mice via CaMKII. Cancer Res. 2010;70:2739-48 pubmed publisher
    ..We show that BCCs induced in conditional Ptch(flox/flox)ERT2(+/-) knockout mice regress with time and show a more differentiated phenotype...
  19. Kim J, Tang J, Gong R, Kim J, Lee J, Clemons K, et al. Itraconazole, a commonly used antifungal that inhibits Hedgehog pathway activity and cancer growth. Cancer Cell. 2010;17:388-99 pubmed publisher
  20. Dijkgraaf G, Alicke B, Weinmann L, Januario T, West K, Modrusan Z, et al. Small molecule inhibition of GDC-0449 refractory smoothened mutants and downstream mechanisms of drug resistance. Cancer Res. 2011;71:435-44 pubmed publisher
    ..Importantly, these HPI resistant MB allografts retained their sensitivity to PI3K inhibition, presenting additional opportunities for the treatment of such tumors...
  21. Kawauchi D, Robinson G, Uziel T, Gibson P, Rehg J, Gao C, et al. A mouse model of the most aggressive subgroup of human medulloblastoma. Cancer Cell. 2012;21:168-80 pubmed publisher
    ..This mouse model should significantly accelerate understanding and treatment of the most aggressive form of medulloblastoma and infers distinct roles for MYC and MYCN in tumorigenesis...
  22. Ahn Y, Sanderson B, Klein O, Krumlauf R. Inhibition of Wnt signaling by Wise (Sostdc1) and negative feedback from Shh controls tooth number and patterning. Development. 2010;137:3221-31 pubmed publisher
    ..These data provide insight into the mechanisms that control Wnt signaling in tooth development and into how crosstalk among signaling pathways controls tooth number and morphogenesis. ..
  23. Hsu S, Zhang X, Yu C, Li Z, Wunder J, Hui C, et al. Kif7 promotes hedgehog signaling in growth plate chondrocytes by restricting the inhibitory function of Sufu. Development. 2011;138:3791-801 pubmed publisher
    ..Furthermore, Kif7 also inhibits Gli transcriptional activity in the chondrocytes when Sufu function is absent. Therefore, Kif7 regulates the activity of Gli transcription factors through both Sufu-dependent and -independent mechanisms...
  24. Aoto K, Trainor P. Co-ordinated brain and craniofacial development depend upon Patched1/XIAP regulation of cell survival. Hum Mol Genet. 2015;24:698-713 pubmed publisher
    ..However, our understanding of how Hh signaling governs cell and tissue survival remains incomplete. Patched1 (Ptch1) is a well-known receptor for Hh ligands and Ptch1 overexpression is associated with cell and tissue-specific ..
  25. Kim J, Aftab B, Tang J, Kim D, Lee A, Rezaee M, et al. Itraconazole and arsenic trioxide inhibit Hedgehog pathway activation and tumor growth associated with acquired resistance to smoothened antagonists. Cancer Cell. 2013;23:23-34 pubmed publisher
    ..Itraconazole and arsenic trioxide, alone or in combination, inhibit the growth of medulloblastoma and basal cell carcinoma in vivo, and prolong survival of mice with intracranial drug-resistant SMO(D477G) medulloblastoma...
  26. Yu T, Yaguchi Y, Echevarria D, Martinez S, Basson M. Sprouty genes prevent excessive FGF signalling in multiple cell types throughout development of the cerebellum. Development. 2011;138:2957-68 pubmed publisher
    ..Taken together, our data demonstrate that FGF signalling levels have to be tightly controlled throughout cerebellar development in order to maintain the normal development of multiple cell types. ..
  27. Nie X, Sun J, Gordon R, Cai C, Xu P. SIX1 acts synergistically with TBX18 in mediating ureteral smooth muscle formation. Development. 2010;137:755-65 pubmed publisher
    ..These findings uncover an essential role for Six1 in establishing a functionally normal ureter and provide new insights into the molecular basis of urinary tract malformations in BOR patients. ..
  28. Xu Q, Guo L, Moore H, Waclaw R, Campbell K, Anderson S. Sonic hedgehog signaling confers ventral telencephalic progenitors with distinct cortical interneuron fates. Neuron. 2010;65:328-40 pubmed publisher
    ..These results indicate that cortical interneuron diversity, a major determinant of cortical function, is critically influenced by differential levels of SHH signaling within the ventral telencephalon. ..
  29. Chen M, Wilson C, Li Y, Law K, Lu C, Gacayan R, et al. Cilium-independent regulation of Gli protein function by Sufu in Hedgehog signaling is evolutionarily conserved. Genes Dev. 2009;23:1910-28 pubmed publisher
    ..Taken together, these studies delineate important aspects of cilium-dependent and cilium-independent Hh signal transduction and provide significant mechanistic insight into Hh signaling in diverse species. ..
  30. Talamillo A, Delgado I, Nakamura T, de Vega S, Yoshitomi Y, Unda F, et al. Role of Epiprofin, a zinc-finger transcription factor, in limb development. Dev Biol. 2010;337:363-74 pubmed publisher
  31. Julian E, Dave R, Robson J, Hallahan A, Wainwright B. Canonical Notch signaling is not required for the growth of Hedgehog pathway-induced medulloblastoma. Oncogene. 2010;29:3465-76 pubmed publisher
    ..a cre recombinase under the control of the GFAP promoter to generate medulloblastoma in mice carrying a conditional Ptc1 allele and introduced a conditional RBP-J allele to ablate canonical Notch signaling...
  32. Miyagawa S, Moon A, Haraguchi R, Inoue C, Harada M, Nakahara C, et al. Dosage-dependent hedgehog signals integrated with Wnt/beta-catenin signaling regulate external genitalia formation as an appendicular program. Development. 2009;136:3969-78 pubmed publisher
    ..Our results provide new insights into the integration of growth factor signaling in the appendicular developmental programs that regulate external genitalia development...
  33. Po A, Ferretti E, Miele E, De Smaele E, Paganelli A, Canettieri G, et al. Hedgehog controls neural stem cells through p53-independent regulation of Nanog. EMBO J. 2010;29:2646-58 pubmed publisher
    ..Our data reveal a mechanism for the function of Hh in the control of stemness that represents a crucial component of an integrated circuitry determining cell fate decision and involved in the maintenance of cancer SCs. ..
  34. Cho S, Kwak S, Woolley T, Lee M, Kim E, Baker R, et al. Interactions between Shh, Sostdc1 and Wnt signaling and a new feedback loop for spatial patterning of the teeth. Development. 2011;138:1807-16 pubmed publisher
  35. Croyle M, Lehman J, O Connor A, Wong S, Malarkey E, Iribarne D, et al. Role of epidermal primary cilia in the homeostasis of skin and hair follicles. Development. 2011;138:1675-85 pubmed publisher
    ..Collectively, these data indicate that epidermal primary cilia may function in stress responses and epidermal homeostasis involving pathways other than those typically associated with primary cilia. ..
  36. Uhmann A, van den Brandt J, Dittmann K, Hess I, Dressel R, Binder C, et al. T cell development critically depends on prethymic stromal patched expression. J Immunol. 2011;186:3383-91 pubmed publisher
    We recently described that T cell specification in mice deficient in the Hedgehog (Hh) receptor Patched (Ptch) is blocked at the level of the common lymphoid progenitor in the bone marrow (BM)...
  37. Sengupta R, Dubuc A, Ward S, Yang L, Northcott P, Woerner B, et al. CXCR4 activation defines a new subgroup of Sonic hedgehog-driven medulloblastoma. Cancer Res. 2012;72:122-32 pubmed publisher
  38. Weatherbee S, Niswander L, Anderson K. A mouse model for Meckel syndrome reveals Mks1 is required for ciliogenesis and Hedgehog signaling. Hum Mol Genet. 2009;18:4565-75 pubmed publisher
    ..Thus the disruption of Hh signaling can explain many, but not all, of the defects caused by loss of Mks1...
  39. Liem K, He M, Ocbina P, Anderson K. Mouse Kif7/Costal2 is a cilia-associated protein that regulates Sonic hedgehog signaling. Proc Natl Acad Sci U S A. 2009;106:13377-82 pubmed publisher
    ..We conclude that Kif7 is a core regulator of Shh signaling that may also act as a ciliary motor. ..
  40. Clement C, Kristensen S, Møllgård K, Pazour G, Yoder B, Larsen L, et al. The primary cilium coordinates early cardiogenesis and hedgehog signaling in cardiomyocyte differentiation. J Cell Sci. 2009;122:3070-82 pubmed publisher
    ..These data support the conclusion that cardiac primary cilia are crucial in early heart development, where they partly coordinate hedgehog signaling. ..
  41. Cain J, Islam E, Haxho F, Chen L, Bridgewater D, Nieuwenhuis E, et al. GLI3 repressor controls nephron number via regulation of Wnt11 and Ret in ureteric tip cells. PLoS ONE. 2009;4:e7313 pubmed publisher
    ..During renal morphogenesis, HH signaling activity, assayed by expression of Ptc1-lacZ, is localized to ureteric cells of the medulla, but is undetectable in the cortex...
  42. Lana Elola E, Tylzanowski P, Takatalo M, Alakurtti K, Veistinen L, Mitsiadis T, et al. Noggin null allele mice exhibit a microform of holoprosencephaly. Hum Mol Genet. 2011;20:4005-15 pubmed publisher
    ..In Noggin(-/-) mice, the expression domains of Shh, as well as the Shh target genes Ptch1 and Gli1, were reduced in the frontonasal region at key stages of early facial development. Using E10...
  43. Hyman J, Firestone A, Heine V, Zhao Y, Ocasio C, Han K, et al. Small-molecule inhibitors reveal multiple strategies for Hedgehog pathway blockade. Proc Natl Acad Sci U S A. 2009;106:14132-7 pubmed publisher
    ..These antagonists therefore constitute a valuable set of chemical tools for interrogating downstream Hh signaling mechanisms and for developing chemotherapies against Hh pathway-related cancers. ..
  44. Allen B, Song J, Izzi L, Althaus I, Kang J, Charron F, et al. Overlapping roles and collective requirement for the coreceptors GAS1, CDO, and BOC in SHH pathway function. Dev Cell. 2011;20:775-87 pubmed publisher
    Secreted Hedgehog (HH) ligands signal through the canonical receptor Patched (PTCH1). However, recent studies implicate three additional HH-binding, cell-surface proteins, GAS1, CDO, and BOC, as putative coreceptors for HH ligands...
  45. Cui C, Chatterjee B, Lozito T, Zhang Z, Francis R, Yagi H, et al. Wdpcp, a PCP protein required for ciliogenesis, regulates directional cell migration and cell polarity by direct modulation of the actin cytoskeleton. PLoS Biol. 2013;11:e1001720 pubmed publisher
    ..Together, these findings provide the first evidence, to our knowledge, that a PCP component required for ciliogenesis can directly modulate the actin cytoskeleton to regulate cell polarity and directional cell migration. ..
  46. Brugmann S, Allen N, James A, Mekonnen Z, Madan E, Helms J. A primary cilia-dependent etiology for midline facial disorders. Hum Mol Genet. 2010;19:1577-92 pubmed publisher
    ..excessive Hedgehog responsiveness in facial mesenchyme, which is accompanied by broader expression domains of Gli1, Ptc and Shh, and reduced expression domains of Gli3...
  47. Miyagawa S, Matsumaru D, Murashima A, Omori A, Satoh Y, Haraguchi R, et al. The role of sonic hedgehog-Gli2 pathway in the masculinization of external genitalia. Endocrinology. 2011;152:2894-903 pubmed publisher
    ..The current study identified hedgehog signaling pathway as a key factor not only for initial development but also for sexually dimorphic development of the external genitalia in coordination with androgen signaling. ..
  48. Ocbina P, Eggenschwiler J, Moskowitz I, Anderson K. Complex interactions between genes controlling trafficking in primary cilia. Nat Genet. 2011;43:547-53 pubmed publisher
  49. Levi B, James A, Nelson E, Brugmann S, Sorkin M, Manu A, et al. Role of Indian hedgehog signaling in palatal osteogenesis. Plast Reconstr Surg. 2011;127:1182-90 pubmed publisher
    ..was used for detecting gene expression during osteogenic differentiation and cellular differentiation (Shh, Ihh, Ptc1, Gli1, Gli2, Gli3, Runx2, Alp, and Col1a1)...
  50. Kim J, Lee J, Kim J, Gardner D, Beachy P. Arsenic antagonizes the Hedgehog pathway by preventing ciliary accumulation and reducing stability of the Gli2 transcriptional effector. Proc Natl Acad Sci U S A. 2010;107:13432-7 pubmed publisher
    ..leukemia (APL); in our studies, ATO inhibited growth of Hh pathway-driven medulloblastoma allografts derived from Ptch+/-p53-/- mice within a range of serum levels comparable to those achieved in treatment of human APL...
  51. Dai D, Zhu H, Wlodarczyk B, Zhang L, Li L, Li A, et al. Fuz controls the morphogenesis and differentiation of hair follicles through the formation of primary cilia. J Invest Dermatol. 2011;131:302-10 pubmed publisher
  52. Cui C, Chatterjee B, Francis D, Yu Q, SanAgustin J, Francis R, et al. Disruption of Mks1 localization to the mother centriole causes cilia defects and developmental malformations in Meckel-Gruber syndrome. Dis Model Mech. 2011;4:43-56 pubmed publisher
    ..On the basis of these results, we hypothesize a role for the B9 domain in mother centriole targeting, a possibility that warrants further future investigations. ..
  53. Mavromatakis Y, Lin W, Metzakopian E, Ferri A, Yan C, Sasaki H, et al. Foxa1 and Foxa2 positively and negatively regulate Shh signalling to specify ventral midbrain progenitor identity. Mech Dev. 2011;128:90-103 pubmed publisher
    ..Altogether, these data indicate that Foxa1 and Foxa2 contribute to the specification of ventral midbrain progenitor identity by regulating Shh signalling in a positive and negative manner. ..
  54. Kerr C, Huang J, Williams T, West Mays J. Activation of the hedgehog signaling pathway in the developing lens stimulates ectopic FoxE3 expression and disruption in fiber cell differentiation. Invest Ophthalmol Vis Sci. 2012;53:3316-30 pubmed publisher
  55. He X, Zhang L, Chen Y, Remke M, Shih D, Lu F, et al. The G protein ? subunit G?s is a tumor suppressor in Sonic hedgehog-driven medulloblastoma. Nat Med. 2014;20:1035-42 pubmed publisher
  56. Liu H, Ermilov A, Grachtchouk M, Li L, Gumucio D, Dlugosz A, et al. Multiple Shh signaling centers participate in fungiform papilla and taste bud formation and maintenance. Dev Biol. 2013;382:82-97 pubmed publisher
    ..We used immunostaining, in situ hybridization and mouse reporter strains for Shh, Ptch1, Gli1 and Gli2-expression and proliferation markers to identify cells that participate in hedgehog signaling...
  57. Bimonte S, De Angelis A, Quagliata L, Giusti F, Tammaro R, Dallai R, et al. Ofd1 is required in limb bud patterning and endochondral bone development. Dev Biol. 2011;349:179-91 pubmed publisher
    ..Our data demonstrate that Ofd1 plays a role in regulating digit number and identity during limb and skeletal patterning increasing insight on the functional role of primary cilia during development. ..
  58. Mao J, Kim B, Rajurkar M, Shivdasani R, McMahon A. Hedgehog signaling controls mesenchymal growth in the developing mammalian digestive tract. Development. 2010;137:1721-9 pubmed publisher
    ..Together, these studies provide new insights into tissue interactions underlying mammalian gastrointestinal organogenesis and disease. ..
  59. Liem K, Ashe A, He M, Satir P, Moran J, Beier D, et al. The IFT-A complex regulates Shh signaling through cilia structure and membrane protein trafficking. J Cell Biol. 2012;197:789-800 pubmed publisher
  60. Lee M, Hatton B, Villavicencio E, Khanna P, Friedman S, Ditzler S, et al. Hedgehog pathway inhibitor saridegib (IPI-926) increases lifespan in a mouse medulloblastoma model. Proc Natl Acad Sci U S A. 2012;109:7859-64 pubmed publisher
    ..The absence of genetic mutations that confer resistance distinguishes saridegib from other Smo inhibitors. ..
  61. Lenton K, James A, Manu A, Brugmann S, Birker D, Nelson E, et al. Indian hedgehog positively regulates calvarial ossification and modulates bone morphogenetic protein signaling. Genesis. 2011;49:784-96 pubmed publisher
    ..In conjunction with the newly recognized roles of Hedgehog deregulation in craniosynostosis, our study defines Ihh as an important positive regulator of cranial bone ossification. ..
  62. Heydeck W, Zeng H, Liu A. Planar cell polarity effector gene Fuzzy regulates cilia formation and Hedgehog signal transduction in mouse. Dev Dyn. 2009;238:3035-42 pubmed publisher
    ..Finally, a significant decrease in cilia was found in Fuz homozygous mutants. In conclusion, Fuz plays an important role in cilia formation, Hh signal transduction, and embryonic development in mammals...
  63. Balaskas N, Ribeiro A, Panovska J, Dessaud E, Sasai N, Page K, et al. Gene regulatory logic for reading the Sonic Hedgehog signaling gradient in the vertebrate neural tube. Cell. 2012;148:273-84 pubmed publisher
    ..Our findings reveal that morphogen interpretation is an emergent property of the architecture of a transcriptional network that provides robustness and reliability to tissue patterning. ..
  64. Cruz C, Ribes V, Kutejova E, Cayuso J, Lawson V, Norris D, et al. Foxj1 regulates floor plate cilia architecture and modifies the response of cells to sonic hedgehog signalling. Development. 2010;137:4271-82 pubmed publisher
  65. Schneider F, Schänzer A, Czupalla C, Thom S, Engels K, Schmidt M, et al. Sonic hedgehog acts as a negative regulator of {beta}-catenin signaling in the adult tongue epithelium. Am J Pathol. 2010;177:404-14 pubmed publisher
    ..We show for the first time that the putative Wnt/beta-catenin targets Shh and JAG2 control beta-catenin signaling in the adult tongue epithelium, a function that is partially lost in lingual SCC. ..
  66. Taniguchi K, Anderson A, Sutherland A, Wotton D. Loss of Tgif function causes holoprosencephaly by disrupting the SHH signaling pathway. PLoS Genet. 2012;8:e1002524 pubmed publisher
    ..These data show for the first time that Tgif1 mutation in mouse contributes to HPE pathogenesis and provide evidence that this is due to disruption of the Shh pathway. ..
  67. Keady B, Samtani R, Tobita K, Tsuchya M, San Agustin J, Follit J, et al. IFT25 links the signal-dependent movement of Hedgehog components to intraflagellar transport. Dev Cell. 2012;22:940-51 pubmed publisher
    ..Thus, IFT function is not restricted to building cilia where signaling occurs, but also plays a separable role in signal transduction events. ..
  68. Berbari N, Kin N, Sharma N, Michaud E, Kesterson R, Yoder B. Mutations in Traf3ip1 reveal defects in ciliogenesis, embryonic development, and altered cell size regulation. Dev Biol. 2011;360:66-76 pubmed publisher
  69. Zhang Z, Wlodarczyk B, Niederreither K, Venugopalan S, Florez S, Finnell R, et al. Fuz regulates craniofacial development through tissue specific responses to signaling factors. PLoS ONE. 2011;6:e24608 pubmed publisher
    ..These data demonstrate a new model for coordination of Hh and Wnt signaling and reveal a Fuz-dependent negative feedback loop controlling Wnt/?-catenin signaling...
  70. Tanori M, Santone M, Mancuso M, Pasquali E, Leonardi S, Di Majo V, et al. Developmental and oncogenic effects of insulin-like growth factor-I in Ptc1+/- mouse cerebellum. Mol Cancer. 2010;9:53 pubmed publisher
    ..Deregulation of the Sonic hedgehog/Patched1 (Shh/Ptc1) signaling pathway predisposes humans and mice to medulloblastoma...
  71. Villani R, Adolphe C, Palmer J, Waters M, Wainwright B. Patched1 inhibits epidermal progenitor cell expansion and basal cell carcinoma formation by limiting Igfbp2 activity. Cancer Prev Res (Phila). 2010;3:1222-34 pubmed publisher
    ..BCC) of the skin is the most common form of cancer, with the majority being caused by mutations in the Patched1 (Ptch1) gene, leading to activation of the Hedgehog (Hh) signaling pathway...
  72. Lee E, Ji H, Ouyang Z, Zhou B, Ma W, Vokes S, et al. Hedgehog pathway-regulated gene networks in cerebellum development and tumorigenesis. Proc Natl Acad Sci U S A. 2010;107:9736-41 pubmed publisher
    ..The results indicate that transformation of normal GNPs into deadly tumor cells is accompanied by a distinct set of Gli-regulated genes and may provide candidates for targeted therapies. ..
  73. Sandell L, Iulianella A, Melton K, Lynn M, Walker M, Inman K, et al. A phenotype-driven ENU mutagenesis screen identifies novel alleles with functional roles in early mouse craniofacial development. Genesis. 2011;49:342-59 pubmed publisher
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