Gli3

Summary

Gene Symbol: Gli3
Description: GLI-Kruppel family member GLI3
Alias: AI854843, AU023367, Bph, GLI3-190, GLI3FL, Pdn, add, transcriptional activator GLI3, GLI3 form of 190 kDa, GLI3 full length protein, anterior digit pattern deformity, brachyphalangy, extra toes, polydactyly Nagoya, zinc finger protein GLI3
Species: mouse

Top Publications

  1. ncbi The graded response to Sonic Hedgehog depends on cilia architecture
    Tamara Caspary
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, NY 10021, USA
    Dev Cell 12:767-78. 2007
  2. pmc Cilium-independent regulation of Gli protein function by Sufu in Hedgehog signaling is evolutionarily conserved
    Miao Hsueh Chen
    Cardiovascular Research Institute, University of California at San Francisco, San Francisco, California 94158, USA
    Genes Dev 23:1910-28. 2009
  3. pmc The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins
    Eric W Humke
    Division of Oncology, Department of Medicine, Stanford University School of Medicine, Stanford, California 94305, USA
    Genes Dev 24:670-82. 2010
  4. ncbi Elimination of a long-range cis-regulatory module causes complete loss of limb-specific Shh expression and truncation of the mouse limb
    Tomoko Sagai
    Mammalian Genetics Laboratory, National Institute of Genetics, Yata 1111 Mishima, Shizuoka ken 411 8540, Japan
    Development 132:797-803. 2005
  5. pmc Mouse Kif7/Costal2 is a cilia-associated protein that regulates Sonic hedgehog signaling
    Karel F Liem
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, NY 10065, USA
    Proc Natl Acad Sci U S A 106:13377-82. 2009
  6. ncbi Hedgehog-regulated processing of Gli3 produces an anterior/posterior repressor gradient in the developing vertebrate limb
    B Wang
    Howard Hughes Medical Institute, Department of Molecular Biology and Genetics, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Cell 100:423-34. 2000
  7. ncbi All mouse ventral spinal cord patterning by hedgehog is Gli dependent and involves an activator function of Gli3
    C Brian Bai
    Howard Hughes Medical Institute and Developmental Genetics Program, Skirball Institute of Biomolecular Medicine, and Department of Cell Biology, New York University School of Medicine, 540 First Avenue, New York, NY 10016, USA
    Dev Cell 6:103-15. 2004
  8. ncbi Hedgehog signalling in the mouse requires intraflagellar transport proteins
    Danwei Huangfu
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, New York 10021, USA
    Nature 426:83-7. 2003
  9. ncbi Dynamic changes in the response of cells to positive hedgehog signaling during mouse limb patterning
    Sohyun Ahn
    Howard Hughes Medical Institute and Developmental Genetics Program, Skirball Institute of Biomolecular Medicine, New York, NY 10016, USA
    Cell 118:505-16. 2004
  10. pmc Kinetics of hedgehog-dependent full-length Gli3 accumulation in primary cilia and subsequent degradation
    Xiaohui Wen
    Genentech, South San Francisco, CA 94080, USA
    Mol Cell Biol 30:1910-22. 2010

Research Grants

Scientific Experts

Detail Information

Publications169 found, 100 shown here

  1. ncbi The graded response to Sonic Hedgehog depends on cilia architecture
    Tamara Caspary
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, NY 10021, USA
    Dev Cell 12:767-78. 2007
    ..Double mutant analysis indicates that Gli3 repressor activity is normal in hnn embryos, but Gli activators are constitutively active at low levels...
  2. pmc Cilium-independent regulation of Gli protein function by Sufu in Hedgehog signaling is evolutionarily conserved
    Miao Hsueh Chen
    Cardiovascular Research Institute, University of California at San Francisco, San Francisco, California 94158, USA
    Genes Dev 23:1910-28. 2009
    ..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...
  3. pmc The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins
    Eric W Humke
    Division of Oncology, Department of Medicine, Stanford University School of Medicine, Stanford, California 94305, USA
    Genes Dev 24:670-82. 2010
    ..We show that the interaction between Gli3 and Suppressor of Fused (Sufu) regulates the formation of either repressor or activator forms of Gli3...
  4. ncbi Elimination of a long-range cis-regulatory module causes complete loss of limb-specific Shh expression and truncation of the mouse limb
    Tomoko Sagai
    Mammalian Genetics Laboratory, National Institute of Genetics, Yata 1111 Mishima, Shizuoka ken 411 8540, Japan
    Development 132:797-803. 2005
    ..These results suggest that the conserved intronic sequence evolved in a common ancestor of fishes and tetrapods to control fin and limb development...
  5. pmc Mouse Kif7/Costal2 is a cilia-associated protein that regulates Sonic hedgehog signaling
    Karel F Liem
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, NY 10065, USA
    Proc Natl Acad Sci U S A 106:13377-82. 2009
    ..We conclude that Kif7 is a core regulator of Shh signaling that may also act as a ciliary motor...
  6. ncbi Hedgehog-regulated processing of Gli3 produces an anterior/posterior repressor gradient in the developing vertebrate limb
    B Wang
    Howard Hughes Medical Institute, Department of Molecular Biology and Genetics, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Cell 100:423-34. 2000
    ..We demonstrate that PKA-dependent processing of vertebrate Gli3 in developing limb similarly generates a potent repressor in a manner antagonized by apparent long-range signaling ..
  7. ncbi All mouse ventral spinal cord patterning by hedgehog is Gli dependent and involves an activator function of Gli3
    C Brian Bai
    Howard Hughes Medical Institute and Developmental Genetics Program, Skirball Institute of Biomolecular Medicine, and Department of Cell Biology, New York University School of Medicine, 540 First Avenue, New York, NY 10016, USA
    Dev Cell 6:103-15. 2004
    ..The full range of Gli activity and its dependence on Hh have not been determined, although the Gli2 activator and Gli3 repressor have been implicated...
  8. ncbi Hedgehog signalling in the mouse requires intraflagellar transport proteins
    Danwei Huangfu
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, New York 10021, USA
    Nature 426:83-7. 2003
    ..Our data show that IFT machinery has an essential and vertebrate-specific role in Hedgehog signal transduction...
  9. ncbi Dynamic changes in the response of cells to positive hedgehog signaling during mouse limb patterning
    Sohyun Ahn
    Howard Hughes Medical Institute and Developmental Genetics Program, Skirball Institute of Biomolecular Medicine, New York, NY 10016, USA
    Cell 118:505-16. 2004
    ..Finally, by fate mapping Shh-responding cells in Gli2 and Gli3 mutant limbs, we demonstrate that a specific level of positive Hh signaling is not required to specify digit ..
  10. pmc Kinetics of hedgehog-dependent full-length Gli3 accumulation in primary cilia and subsequent degradation
    Xiaohui Wen
    Genentech, South San Francisco, CA 94080, USA
    Mol Cell Biol 30:1910-22. 2010
    ..By generating antibodies capable of detecting endogenous pathway transcription factors Gli2 and Gli3, we monitored their kinetics of accumulation in cilia upon Hh stimulation...
  11. ncbi Ftm is a novel basal body protein of cilia involved in Shh signalling
    Jeanette Vierkotten
    Institut für Entwicklungs und Molekularbiologie der Tiere EMT, Heinrich Heine Universitat, 40225 Dusseldorf, Germany
    Development 134:2569-77. 2007
    ..The loss of Ftm affects the ratio of Gli3 activator to Gli3 repressor, suggesting an involvement of Ftm in Shh signalling...
  12. ncbi Cholesterol modification of sonic hedgehog is required for long-range signaling activity and effective modulation of signaling by Ptc1
    P M Lewis
    Department of Molecular and Cellular Biology, The Biolabs, Harvard University, 16 Divinity Avenue, Cambridge, MA 02138, USA
    Cell 105:599-612. 2001
    ..Thus, cholesterol modification is essential for the normal range of signaling. It also appears to be necessary for appropriate modulation of signaling by the Shh receptor, Ptc1...
  13. ncbi Mouse GLI3 regulates Fgf8 expression and apoptosis in the developing neural tube, face, and limb bud
    Kazushi Aoto
    Molecular Neuropathology Group, Brain Science Institute, The Institute of Physical and Chemical Research RIKEN, 2 1 Hirosawa, Wako, Saitama 351 0198, Japan
    Dev Biol 251:320-32. 2002
    The zinc finger transcription factor GLI3 is considered a repressor of vertebrate Hedgehog (Hh) signaling...
  14. ncbi Diminished Sonic hedgehog signaling and lack of floor plate differentiation in Gli2 mutant mice
    Q Ding
    Program in Developmental Biology and Division of Endocrinology, Research Institute, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
    Development 125:2533-43. 1998
    ..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 can occur in the absence of floor plate induction...
  15. pmc Cilia and Hedgehog responsiveness in the mouse
    Danwei Huangfu
    Developmental Biology Program, Sloan Kettering Institute, 1275 York Avenue, New York, NY 10021, USA
    Proc Natl Acad Sci U S A 102:11325-30. 2005
    ..patterning phenotype than Smo mutants, because Ift172, but not Smo, is required for proteolytic processing of Gli3 to its repressor form...
  16. ncbi Loss of the Tg737 protein results in skeletal patterning defects
    Qihong Zhang
    The University of Alabama at Birmingham, Department of Cell Biology, Birmingham, Alabama 35294, USA
    Dev Dyn 227:78-90. 2003
    ..Collectively, the data argue for a dosage effect of Tg737 on the limb phenotypes and that the polydactyly is independent of Shh misexpression...
  17. ncbi Expression of three mouse homologs of the Drosophila segment polarity gene cubitus interruptus, Gli, Gli-2, and Gli-3, in ectoderm- and mesoderm-derived tissues suggests multiple roles during postimplantation development
    C C Hui
    Division of Molecular and Developmental Biology, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada
    Dev Biol 162:402-13. 1994
    ..Consistent with this hypothesis, a naturally occurring Gli-3 mutation, the mouse extra-toes mutant; shows defects in both mesoderm- and ectoderm-derived tissues...
  18. ncbi Gli3 acts as a repressor downstream of Ihh in regulating two distinct steps of chondrocyte differentiation
    Lydia Koziel
    Max Planck Institute for Molecular Genetics, Berlin, Germany
    Development 132:5249-60. 2005
    ..signal is translated in the different target tissues, we analyzed the role of the zinc-finger transcription factor Gli3, which acts downstream of hedgehog signals in other organs...
  19. doi Gli3-deficient mice exhibit cleft palate associated with abnormal tongue development
    Xi Huang
    Department of Cell and Developmental Biology, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA
    Dev Dyn 237:3079-87. 2008
    ..We observed a high incidence of palate clefting in mice deficient in Gli3, known for its role as a repressor in the absence of Shh signaling...
  20. doi A homeodomain feedback circuit underlies step-function interpretation of a Shh morphogen gradient during ventral neural patterning
    Madelen Lek
    Department of Cell and Molecular Biology, Karolinska Institutet, Stockholm, Sweden
    Development 137:4051-60. 2010
    ..These data establish that dynamic, non-graded changes in responding cells are essential for Shh morphogen interpretation, and provide a rationale to explain mechanistically the phenomenon of cellular memory of morphogen exposure...
  21. pmc A novel murine allele of Intraflagellar Transport Protein 172 causes a syndrome including VACTERL-like features with hydrocephalus
    Joshua M Friedland-Little
    Department of Pediatrics, The University of Chicago, 900 East 57th Street, Chicago, IL 60637, USA
    Hum Mol Genet 20:3725-37. 2011
    ..We propose a model in which mutation of Ift172 results in a specific class of abnormal cilia, causing disrupted Hh signaling while maintaining L-R axis determination, and resulting in the VACTERL-H phenotype...
  22. pmc Fuz regulates craniofacial development through tissue specific responses to signaling factors
    Zichao Zhang
    Institute of Biosciences and Technology, Texas A and M Health Science Center, Houston, Texas, United States of America
    PLoS ONE 6:e24608. 2011
    ..These data demonstrate a new model for coordination of Hh and Wnt signaling and reveal a Fuz-dependent negative feedback loop controlling Wnt/β-catenin signaling...
  23. pmc A primary cilia-dependent etiology for midline facial disorders
    Samantha A Brugmann
    Department of Surgery, Division of Plastic and Reconstructive Surgery, Stanford University, Stanford, CA 94305, USA
    Hum Mol Genet 19:1577-92. 2010
    ..which is accompanied by broader expression domains of Gli1, Ptc and Shh, and reduced expression domains of Gli3. Furthermore, broader domains of Gli1 expression correspond to areas of enhanced neural crest cell proliferation in ..
  24. pmc A mechanism for vertebrate Hedgehog signaling: recruitment to cilia and dissociation of SuFu-Gli protein complexes
    Hanna Tukachinsky
    Department of Cell Biology, Harvard Medical School, Boston, MA 02115, USA
    J Cell Biol 191:415-28. 2010
    ..Our results support a simple mechanism in which Hh signals at vertebrate cilia cause dissociation of inactive SuFu-Gli complexes, a process inhibited by PKA...
  25. pmc Mouse hitchhiker mutants have spina bifida, dorso-ventral patterning defects and polydactyly: identification of Tulp3 as a novel negative regulator of the Sonic hedgehog pathway
    Victoria L Patterson
    Mammalian Genetics Unit, MRC Harwell, Harwell Science and Innovation Campus, Oxon, UK
    Hum Mol Genet 18:1719-39. 2009
    ..downstream of Shh and Smoothened (Smo) in neural tube patterning and exhibits a genetic interaction with Gli3 in limb development...
  26. ncbi Extra-toes: anew mutant gene causing multiple abnormalities in the mouse
    D R Johnson
    J Embryol Exp Morphol 17:543-81. 1967
  27. ncbi Unique functions of Sonic hedgehog signaling during external genitalia development
    R Haraguchi
    Center for Animal Resources and Development CARD and Graduate School of Molecular and Genomic Pharmacy, Kumamoto University, Honjo 2 2 1, Kumamoto 860 0811, Japan
    Development 128:4241-50. 2001
    ..These results suggest a dual mode of Shh function, first by the regulation of initiating GT outgrowth, and second, by subsequent GT differentiation...
  28. ncbi High-resolution mapping of the Gli3 mutation extra-toes reveals a 51.5-kb deletion
    Thomas M Maynard
    Department of Cell and Molecular Physiology, UNC Neuroscience Center, CB 7545, School of Medicine, University of North Carolina, Chapel Hill, North Carolina 27599, USA
    Mamm Genome 13:58-61. 2002
  29. doi Mouse Shh is required for prechordal plate maintenance during brain and craniofacial morphogenesis
    Kazushi Aoto
    Molecular Neuropathology Group, Brain Science Institute, RIKEN, 2 1 Hirosawa, Wako, Saitama 351 0198, Japan
    Dev Biol 327:106-20. 2009
    ..These findings provide new insight into the mechanism underlying HPE pathoetiology during dynamic brain and craniofacial morphogenesis...
  30. ncbi Evidence for the involvement of the Gli gene family in embryonic mouse lung development
    J C Grindley
    Department of Cell Biology, Vanderbilt University Medical Center, Nashville, Tennessee, 37232 2175, USA
    Dev Biol 188:337-48. 1997
    Murine Gli, Gli2, and Gli3 are zinc finger genes related to Drosophila cubitus interuptus, a component of the hedgehog signal transduction pathway...
  31. pmc Sonic hedgehog signaling regulates Gli2 transcriptional activity by suppressing its processing and degradation
    Yong Pan
    Weill Medical College of Cornell University, 1300 York Avenue, Room W404, New York, NY 10021, USA
    Mol Cell Biol 26:3365-77. 2006
    Gli2 and Gli3 are the primary transcription factors that mediate Sonic hedgehog (Shh) signals in the mouse. Gli3 mainly acts as a transcriptional repressor, because the majority of full-length Gli3 protein is proteolytically processed...
  32. doi Shh dependent and independent maintenance of basal midbrain
    Ariadna Perez-Balaguer
    Instituto de Neurociencias de Alicante, CSIC and Universidad Miguel Hernandez, Sant Joan d Alacant, Spain
    Mech Dev 126:301-13. 2009
    ..Based on our results and recently published data, we suggest the existence of a specific genetic pathway for the specification of the mesencephalic red nucleus. Foxa2 could be the candidate gene that might control this genetic pathway...
  33. ncbi A binding site for Gli proteins is essential for HNF-3beta floor plate enhancer activity in transgenics and can respond to Shh in vitro
    H Sasaki
    Laboratory of Developmental Biology, Osaka University, Suita, Japan
    Development 124:1313-22. 1997
    ..Gli expression is restricted to the ventral part, while Gli2 and Gli3 are expressed throughout the neural tube and dorsally, respectively...
  34. ncbi Polydactyly and ectopic ZPA formation in Alx-4 mutant mice
    S Qu
    Department of Biochemistry, Vanderbilt University School of Medicine, Nashville, TN 37232, USA
    Development 124:3999-4008. 1997
    ..of other candidate regulators of anterior-posterior positional information in the limb bud, including HoxB8 and Gli3, is not altered in Alx-4 mutant embryos...
  35. ncbi Specific and redundant functions of Gli2 and Gli3 zinc finger genes in skeletal patterning and development
    R Mo
    Program in Developmental Biology and Division of Endocrinology, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
    Development 124:113-23. 1997
    ..Interestingly, Gli2 and Gli3 (C.-c. Hui and A. L. Joyner (1993). Nature Genet...
  36. doi Sonic Hedgehog mediator Gli2 regulates bladder mesenchymal patterning
    Wei Cheng
    Division of Paediatric Surgery, Department of Paediatrics, Monash University, Melbourne, Australia
    J Urol 180:1543-50. 2008
    ..Current evidence suggests that Shh (R&D Systems) is an epithelial signal regulating bladder development, although the mechanism of the regulation is still unclear. We examined the regulation of bladder mesenchymal development...
  37. pmc Primary cilia regulate Shh activity in the control of molar tooth number
    Atsushi Ohazama
    Department of Craniofacial Development, Dental Institute, King s College London, London SE1 9RT, UK
    Development 136:897-903. 2009
    ..Strikingly, the ectopic teeth adopt a size and shape characteristic of premolars, a tooth type that was lost in mice around 50-100 million years ago...
  38. pmc Paracrine Hedgehog signaling in stomach and intestine: new roles for hedgehog in gastrointestinal patterning
    Asa Kolterud
    Department of Cell and Developmental Biology, University of Michigan Medical School, Ann Arbor, Michigan 48109 2200, USA
    Gastroenterology 137:618-28. 2009
    ..We profiled cellular Hedgehog response patterns from embryonic day 10.5 (E10.5) to adult in murine antrum, pyloric region, small intestine, and colon...
  39. pmc Distinct roles of Hand2 in initiating polarity and posterior Shh expression during the onset of mouse limb bud development
    Antonella Galli
    Developmental Genetics, Department of Biomedicine, University of Basel, Basel, Switzerland
    PLoS Genet 6:e1000901. 2010
    ..studies indicate that Hand2 and Hoxd13 can efficiently transactivate gene expression via the ZRS, while the Gli3 repressor isoform interferes with this positive transcriptional regulation...
  40. ncbi Multigenic control of the localization of the zone of polarizing activity in limb morphogenesis in the mouse
    H Masuya
    Mammalian Genetics Laboratory, National Institute of Genetics, Mishima, Japan
    Dev Biol 182:42-51. 1997
    ..We studied the spatial and temporal pattern of expression of the Gli3 gene, which is affected in a loss-of-function type of mutation, Xt1...
  41. ncbi Genetics of shoulder girdle formation: roles of Tbx15 and aristaless-like genes
    Sanne Kuijper
    Hubrecht Laboratory, The Netherlands Institute for Developmental Biology, Uppsalalaan 8, 3584CT Utrecht, The Netherlands
    Development 132:1601-10. 2005
    ..We have studied the impact of Tbx15, Gli3, Alx4 and related genes on formation of the skeletal elements of the mouse shoulder and pelvic girdles...
  42. ncbi Essential function of Gli2 and Gli3 in the formation of lung, trachea and oesophagus
    J Motoyama
    Programs in Developmental Biology, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
    Nat Genet 20:54-7. 1998
    ..In mice, three zinc-finger transcription factors, Gli1, Gli2 and Gli3, have been implicated in the transduction of Shh signal...
  43. ncbi Gli2 and Gli3 have redundant and context-dependent function in skeletal muscle formation
    Aileen McDermott
    Department of Biomedical Science, University of Sheffield, Firth Court, Western Bank, Sheffield S10 2TN, UK
    Development 132:345-57. 2005
    ..We report that Gli genes are differentially expressed in the mouse somite. Gli2 and Gli3 are essential for Gli1 expression in somites, establishing Gli2 and Gli3 as primary mediators and Gli1 as a ..
  44. ncbi Unique and complimentary activities of the Gli transcription factors in Hedgehog signaling
    Robert J Lipinski
    Molecular and Environmental Toxicology Center, Madison, WI 53705 222, USA
    Exp Cell Res 312:1925-38. 2006
    ..Gli2 loss-of-function was associated with diminished Shh-induced target gene expression, while Gli3 loss-of-function was associated with increased basal and Shh-induced target gene expression...
  45. ncbi FGF signalling generates ventral telencephalic cells independently of SHH
    Grigoriy Gutin
    Departments of Neuroscience and Molecular Genetics, Albert Einstein College of Medicine, 1410 Pelham Parkway South, Bronx, NY 10461, USA
    Development 133:2937-46. 2006
    ..however, has recently been questioned because loss of the Shh gene has little effect on ventral development if the Gli3 gene is also mutant. Consequently, another ventral determinant must exist...
  46. pmc Kinesin-2 controls development and patterning of the vertebrate skeleton by Hedgehog- and Gli3-dependent mechanisms
    Elona Kolpakova-Hart
    Department of Developmental Biology, Harvard School of Dental Medicine, Boston, Massachusetts 02115, USA
    Dev Biol 309:273-84. 2007
    ..We show that in Kif3a-deficient mesenchymal tissues both the repressor function of Gli3 transcription factor and the activation of the Shh transcriptional targets Ptch and Gli1 are compromised...
  47. ncbi Evidence for genetic control of Sonic hedgehog by Gli3 in mouse limb development
    D Buscher
    Institut fur Molekularbiologie, Medizinische Hochschule Hannover, Germany
    Mech Dev 62:175-82. 1997
    ..In the polydactyly mouse mutant extra toes (Xt), possessing a null mutation of Gli3, Shh is additionally expressed in the anterior region of the limb bud...
  48. ncbi Control of cell pattern in the neural tube by the zinc finger transcription factor and oncogene Gli-1
    M Hynes
    Department of Neuroscience, Genentech, Inc, South San Francisco, California 94080, USA
    Neuron 19:15-26. 1997
    ..These findings demonstrate that GLI-1 can reproduce the cell patterning actions of Shh in the developing nervous system and provide support for the hypothesis that it is a mediator of the Shh signal in vertebrates...
  49. ncbi Gli3 (Xt) and formin (ld) participate in the positioning of the polarising region and control of posterior limb-bud identity
    A Zuniga
    EMBL, Meyerhofstrasse 1, D 69117 Heidelberg, Germany
    Development 126:13-21. 1999
    ..The unique and shared functions of Gli3 and formin in these determinative events were genetically analyzed using single and double homozygous Extra-toes (..
  50. ncbi Defects in mouse mammary gland development caused by conditional haploinsufficiency of Patched-1
    M T Lewis
    Department of Biology, Sinsheimer Laboratories, University of California, Santa Cruz, CA 95064, USA
    Development 126:5181-93. 1999
    ..Data demonstrate a critical mammary role for at least one component of the hedgehog signaling network and suggest that Ihh is the primary hedgehog gene active in the gland...
  51. ncbi Sonic hedgehog regulates growth and morphogenesis of the tooth
    H R Dassule
    Department of Molecular and Cellular Biology, The Biolabs, Divinity Avenue, Cambridge, MA 02138, USA
    Development 127:4775-85. 2000
    ..These studies demonstrate that Shh regulates growth and determines the shape of the tooth. However, Shh signaling is not essential for differentiation of ameloblasts or odontoblasts...
  52. ncbi Manifestation of the limb prepattern: limb development in the absence of sonic hedgehog function
    C Chiang
    Department of Cell Biology, Vanderbilt University Medical Center, 1161 21st Avenue South, Nashville, Tennessee 37232, USA
    Dev Biol 236:421-35. 2001
    ..According to this model, the limb bud signaling centers, including the zone of polarizing activity (ZPA) acting through Shh, are required to elaborate upon the axial information provided by the native limb field prepattern...
  53. pmc Mutual genetic antagonism involving GLI3 and dHAND prepatterns the vertebrate limb bud mesenchyme prior to SHH signaling
    Pascal te Welscher
    Department of Developmental Biology, Faculty of Biology, Utrecht University, 3584CH Utrecht, The Netherlands
    Genes Dev 16:421-6. 2002
    ..We show that the transcriptional repressor GLI3 restricts dHAND expression to posterior mesenchyme prior to activation of SHH signaling in mouse limb buds...
  54. ncbi Mouse Twist is required for fibroblast growth factor-mediated epithelial-mesenchymal signalling and cell survival during limb morphogenesis
    Aimee Zuniga
    Department of Developmental Biology, Faculty of Biology, Utrecht University, Padualaan 8, 3584 CH Utrecht, The Netherlands
    Mech Dev 114:51-9. 2002
    ..Finally, Twist function, most likely by regulating FGF signalling, is required for cell survival as apoptotic cells are detected in posterior and distal limb bud mesenchyme...
  55. ncbi Shh signaling within the dental epithelium is necessary for cell proliferation, growth and polarization
    Amel Gritli-Linde
    Department of Oral Biochemistry, Sahlgrenska Academy at Goteborg University, Sweden
    Development 129:5323-37. 2002
    ..Furthermore, we provide evidence that Shh signaling between ameloblasts and the overlying stratum intermedium may involve subcellular localization of Patched 2 and Gli1 mRNAs, both of which are targets of Shh signaling in these cells...
  56. ncbi Mouse intraflagellar transport proteins regulate both the activator and repressor functions of Gli transcription factors
    Aimin Liu
    Department of Pediatrics, University of Colorado at Denver and Health Sciences Center, Mailstop 8322, Box 6511, Aurora, CO 80045, USA
    Development 132:3103-11. 2005
    ..domains of multiple posteriorly restricted genes are expanded anteriorly in the mutant limbs, similar to loss of Gli3 transcriptional repressor function...
  57. ncbi Gli3 and Plzf cooperate in proximal limb patterning at early stages of limb development
    Maria Barna
    Weill Graduate School of Medical Sciences, Cornell University, New York, New York 10021, USA
    Nature 436:277-81. 2005
    ..Here we identify a genetic interaction between Gli3 (GLI-Kruppel family member 3) and Plzf (promyelocytic leukaemia zinc finger, also known as Zbtb16 and Zfp145), ..
  58. ncbi Mouse Rab23 regulates hedgehog signaling from smoothened to Gli proteins
    Jonathan T Eggenschwiler
    Developmental Biology Program, Sloan Kettering Institute, New York, NY 10021, USA
    Dev Biol 290:1-12. 2006
    ..Double mutant analysis indicates that the primary target of Rab23 is the Gli2 activator and that Rab23 and Gli3 repressor have additive effects on patterning...
  59. ncbi Netrin 1 regulates ventral tangential migration of guidepost neurons in the lateral olfactory tract
    Takahiko Kawasaki
    Division of Brain Function, National Institute of Genetics, Graduate University for Advanced Studies, SOKENDAI, Yata 1111, Mishima 411 8540, Japan
    Development 133:845-53. 2006
    ..These results suggest that netrin 1 regulates the migration of lot cells and LOT projections, possibly by ensuring the correct distribution of these guidepost neurons...
  60. ncbi Pbx1/Pbx2 requirement for distal limb patterning is mediated by the hierarchical control of Hox gene spatial distribution and Shh expression
    Terence D Capellini
    Department of Cell and Developmental Biology, Cornell University Weill Medical School, New York, NY 10021, USA
    Development 133:2263-73. 2006
    ....
  61. ncbi Interactions between HOXD and Gli3 genes control the limb apical ectodermal ridge via Fgf10
    Jozsef Zakany
    Department of Zoology and Animal Biology and National Research Centre Frontiers in Genetics, University of Geneva, Sciences III, Quai Ernest Ansermet 30, 1211 Geneva 4, Switzerland
    Dev Biol 306:883-93. 2007
    ..Here, we describe antagonistic interactions between posterior Hoxd genes and Gli3, suggesting that the latter product protects the AER from the deleterious effect of the formers, and we present ..
  62. ncbi Wnt canonical pathway restricts graded Shh/Gli patterning activity through the regulation of Gli3 expression
    Roberto Alvarez-Medina
    Instituto de Biologia Molecular de Barcelona, CSIC, Parc Cientific de Barcelona, C Josep Samitier 1 5, Barcelona 08028, Spain
    Development 135:237-47. 2008
    ..Additionally, we show that Gli3 expression is controlled by Wnt activity...
  63. doi Analysis of early ventral telencephalic defects in mice lacking functional Gli3 protein
    Tian Yu
    Centres for Integrative Physiology and Neuroscience Research, University of Edinburgh, Edinburgh, United Kingdom
    J Comp Neurol 512:613-27. 2009
    The transcription factor Gli3 is expressed throughout developing telencephalon. Previous studies have focused on Gli3's role in dorsal telencephalon, which is greatly reduced in size in Gli3(Xt/Xt) mutants...
  64. pmc Shh and Gli3 activities are required for timely generation of motor neuron progenitors
    Saeock Oh
    Department of Cell and Developmental Biology, Center for Molecular Neuroscience, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    Dev Biol 331:261-9. 2009
    ..Genetic studies of Shh(-/-);Gli3(-/-) double mutants indicated that the inhibition of Gli3 repressor activity by Shh is sufficient for the ..
  65. ncbi Deletion of GLI3 supports the homology of the human Greig cephalopolysyndactyly syndrome (GCPS) and the mouse mutant extra toes (Xt)
    A Vortkamp
    Institut für Humangenetik der Philipps Universität, Marburg, FRG
    Mamm Genome 3:461-3. 1992
  66. ncbi GLI3 zinc-finger gene interrupted by translocations in Greig syndrome families
    A Vortkamp
    Institut fur Humangenetik, Philipps Universitat, Marburg, Germany
    Nature 352:539-40. 1991
    ..Here we show that two of the three translocations interup the GLI3 gene, a zinc-finger gene of the GLI-Krüppel family already localized to 7p13 (refs 5, 6)...
  67. ncbi Gli2 is required for induction of floor plate and adjacent cells, but not most ventral neurons in the mouse central nervous system
    M P Matise
    Developmental Genetics Program and Howard Hughes Medical Institute, and Department of Cell Biology and Physiology and Neuroscience, NYU Medical Center, New York, NY 10016, USA
    Development 125:2759-70. 1998
    ....
  68. ncbi Sonic hedgehog regulates branching morphogenesis in the mammalian lung
    C V Pepicelli
    Department of Molecular and Cellular Biology, Harvard University, Cambridge, Massachusetts 02138, USA
    Curr Biol 8:1083-6. 1998
    ..In addition, the transcription of several mesenchymally expressed downstream targets of Shh is abolished. These results highlight the importance of epithelially derived Shh in regulating branching morphogenesis of the lung...
  69. ncbi Sonic Hedgehog-induced activation of the Gli1 promoter is mediated by GLI3
    P Dai
    Laboratory of Molecular Genetics, Tsukuba Life Science Center, RIKEN, 3 1 1 Koyadai, Tsukuba, Ibaraki 305 0074, Japan
    J Biol Chem 274:8143-52. 1999
    ..Here, we report distinct roles for GLI1 and GLI3 in Sonic hedgehog (Shh) signaling...
  70. ncbi Dorsoventral patterning of the telencephalon is disrupted in the mouse mutant extra-toes(J)
    S Tole
    Department of Neurobiology, University of Chicago, Chicago, Illinois, 60637, USA
    Dev Biol 217:254-65. 2000
    ..patterning in the extra-toes (J) (Xt(J)) mouse mutant, which carries a deletion in the Gli family member Gli3. We report that dorsoventral patterning of the telencephalon is dramatically disrupted in the Xt(J) mutant...
  71. ncbi Genomic organization, chromosomal assignment, and expression analysis of the mouse suppressor of fused gene (Sufu) coding a Gli protein partner
    D Simon-Chazottes
    Unite de Genetique des Mammiferes, Institut Pasteur, 25 rue du Dr Roux, 75724 Paris Cedex 15, France
    Mamm Genome 11:614-21. 2000
    ..Sufu binds with all three Gli proteins, with different affinities. This report, in conjunction with recent studies, points out the importance of Sufu in mouse embryonic development...
  72. ncbi Murine models of VACTERL syndrome: Role of sonic hedgehog signaling pathway
    P C Kim
    Programs in Infection, Immunity, Injury and Repair and Developmental Biology, Research Institute, The Hospital for Sick Children, Department of Surgery, University of Toronto, Toronto, Ontario, Canada
    J Pediatr Surg 36:381-4. 2001
    ..The etiology, embryology, and pathogenesis of the VACTERL syndrome are not known. The authors report here new mouse models of VACTERL syndrome involving the Sonic hedgehog (Shh) signaling pathway...
  73. ncbi Progression of vertebrate limb development through SHH-mediated counteraction of GLI3
    Pascal te Welscher
    Department of Developmental Biology, Faculty of Biology, Utrecht University, Padualaan 8, NL 3584 CH Utrecht, Netherlands
    Science 298:827-30. 2002
    ..Here, we report that the polydactyly of Gli3-deficient mice arises independently of SHH signaling...
  74. pmc Ski is involved in transcriptional regulation by the repressor and full-length forms of Gli3
    Ping Dai
    Laboratory of Molecular Genetics, RIKEN Tsukuba Institute, Ibaraki, Japan
    Genes Dev 16:2843-8. 2002
    Transcription factor Glioblastoma-3 (Gli3) is cleaved in the anterior region of the limb bud to generate its repressor form...
  75. pmc A direct requirement for Hedgehog signaling for normal specification of all ventral progenitor domains in the presumptive mammalian spinal cord
    Mark Wijgerde
    Department of Molecular and Cellular Biology, The Biolabs, Harvard University, Cambridge, Massachusetts 02138, USA
    Genes Dev 16:2849-64. 2002
    ..Comparative studies of Shh, Smo, and Gli3 single and compound mutants reveal that Hh signaling acts in part to specify neural cell identity by counteracting ..
  76. pmc Sonic hedgehog-dependent activation of Gli2 is essential for embryonic hair follicle development
    Pleasantine Mill
    Program in Developmental Biology, The Hospital for Sick Children, and Department of Molecular and Medical Genetics, University of Toronto, Toronto, Ontario M5G 1X8, Canada
    Genes Dev 17:282-94. 2003
    ..Together, our results suggest that Shh-dependent Gli2 activation plays a critical role in epithelial homeostasis by promoting proliferation through the transcriptional control of cell cycle regulators...
  77. ncbi A disrupted balance between Bmp/Wnt and Fgf signaling underlies the ventralization of the Gli3 mutant telencephalon
    Stefanie Kuschel
    Institute for Animal Developmental and Molecular Biology, Heinrich Heine University, D 40225 Dusseldorf, Germany
    Dev Biol 260:484-95. 2003
    ..we report on telencephalic development in Pdn mutant mice, which carry an integration of a retrotransposon in the Gli3 locus...
  78. ncbi Early developmental arrest of mammalian limbs lacking HoxA/HoxD gene function
    Marie Kmita
    Department of Zoology and Animal Biology and National Research Centre Frontiers in Genetics, University of Geneva, Sciences III, Quai Ernest Ansermet 30, 1211 Geneva 4, Switzerland
    Nature 435:1113-6. 2005
    ..Accordingly, these mutant limbs may be reminiscent of an ancestral trunk extension, related to that proposed for arthropods...
  79. ncbi Pax9 and Jagged1 act downstream of Gli3 in vertebrate limb development
    Edwina McGlinn
    Institute for Molecular Bioscience, The University of Queensland, Brisbane, QLD 4072, Australia
    Mech Dev 122:1218-33. 2005
    From early in limb development the transcription factor Gli3 acts to define boundaries of gene expression along the anterior-posterior (AP) axis, establishing asymmetric patterns required to provide positional information...
  80. ncbi Molecular analysis of coordinated bladder and urogenital organ formation by Hedgehog signaling
    Ryuma Haraguchi
    Center for Animal Resources and Development, CARD and Pharmaceutical Sciences, Kumamoto University, Honjo 2 2 1, Kumamoto, Japan
    Development 134:525-33. 2007
    ..Thus, the mesenchymal precursors for multiple urogenital structures derive from peri-cloacal mesenchyme and the coordination of urogenital organ formation from these precursors is orchestrated by Shh signals...
  81. ncbi Opposing gradients of Gli repressor and activators mediate Shh signaling along the dorsoventral axis of the inner ear
    Jinwoong Bok
    National Institute on Deafness and Other Communication Disorders, Rockville, MD 20850, USA
    Development 134:1713-22. 2007
    ..regulates inner ear development, we examined embryos with various combinations of mutant alleles for Shh, Gli2 and Gli3. Our study shows that Gli3 repressor (R) is required for patterning dorsal inner ear structures, whereas Gli ..
  82. ncbi Hoxd and Gli3 interactions modulate digit number in the amniote limb
    Rushikesh Sheth
    Departamento de Anatomia y Biologia Celular, Universidad de Cantabria, 39011, Santander, Spain
    Dev Biol 310:430-41. 2007
    ..SHH signaling prevents the processing of GLI3 into a short form that functions as a strong transcriptional repressor...
  83. doi Altered signaling pathway in the dysmorphogenesis of telencephalon in the Gli3 depressed mouse embryo, Pdn/Pdn
    Etsuko Ueta
    School of Health Science, Faculty of Medicine, Tottori University, Yonago, Japan
    Congenit Anom (Kyoto) 48:74-80. 2008
    The responsible gene of genetic polydactyly/arhinencephaly mouse (Pdn/Pdn) is Gli3. Pdn/Pdn exhibits absence of the olfactory bulb, suggesting telencephalic dysmorphogenesis...
  84. doi The mammalian Cos2 homolog Kif7 plays an essential role in modulating Hh signal transduction during development
    Setsu Endoh-Yamagami
    Department of Molecular Biology, Genentech, Inc, 1 DNA Way, South San Francisco, CA 94080, USA
    Curr Biol 19:1320-6. 2009
    ..We also demonstrate a requirement for Kif7 in the efficient localization of Gli3 to cilia in response to Hh and for the processing of Gli3 to its repressor form...
  85. doi Planar cell polarity effector gene Fuzzy regulates cilia formation and Hedgehog signal transduction in mouse
    Westley Heydeck
    Department of Biology, Eberly College of Science, The Pennsylvania State University, University Park, Pennsylvania 16802, USA
    Dev Dyn 238:3035-42. 2009
    ..Fuz mutants also exhibit abnormal Hedgehog (Hh) signaling and inefficient proteolytic processing of Gli3. Finally, a significant decrease in cilia was found in Fuz homozygous mutants...
  86. pmc Dosage-dependent hedgehog signals integrated with Wnt/beta-catenin signaling regulate external genitalia formation as an appendicular program
    Shinichi Miyagawa
    Institute of Molecular Embryology and Genetics, Global COE Cell Fate Regulation Research and Education Unit, Kumamoto University, Kumamoto 860 0811, Japan
    Development 136:3969-78. 2009
    ..Our results provide new insights into the integration of growth factor signaling in the appendicular developmental programs that regulate external genitalia development...
  87. pmc GLI3 repressor controls functional development of the mouse ureter
    Jason E Cain
    Program in Developmental and Stem Cell Biology, Hospital for Sick Children, Toronto, Ontario, Canada
    J Clin Invest 121:1199-206. 2011
    ..and cell differentiation by promoting GLI-dependent transcriptional activation and by inhibiting the processing of GLI3 to a transcriptional repressor...
  88. pmc The IFT-A complex regulates Shh signaling through cilia structure and membrane protein trafficking
    Karel F Liem
    Developmental Biology Program, Sloan Kettering Institute, New York, NY 10065, USA
    J Cell Biol 197:789-800. 2012
    ....
  89. ncbi The development of the eye in homozygotes of the mouse mutant Extra-toes
    T Franz
    Abteilung fur Neuroanatomie, Universitäts Krankenhaus Eppendorf Hamburg, Federal Republic of Germany
    Anat Embryol (Berl) 184:355-61. 1991
    ....
  90. ncbi Brachyphalangy, an allele of extra-toes in the mouse
    D R Johnson
    Genet Res 13:275-80. 1969
  91. ncbi A duplicated zone of polarizing activity in polydactylous mouse mutants
    H Masuya
    Mammalian Genetics Laboratory, National Institute of Genetics, Mishima, Japan
    Genes Dev 9:1645-53. 1995
    ..Here we report that a new mouse mutant, Recombination induced mutant 4 (Rim4), and two old mutants, Hemimelic extra toes (Hx) and Extra toes (Xt), exhibit mirror-image duplications of the skeletal pattern of the digits...
  92. ncbi The mouse mutant Polydactyly Nagoya (Pdn) defines a novel allele of the zinc finger gene Gli3
    T Schimmang
    European Molecular Biology Laboratory, Heidelberg, Germany
    Mamm Genome 5:384-6. 1994
  93. ncbi A mouse model of greig cephalopolysyndactyly syndrome: the extra-toesJ mutation contains an intragenic deletion of the Gli3 gene
    C C Hui
    Division of Molecular and Developmental Biology, Samuel Lunenfield Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada
    Nat Genet 3:241-6. 1993
    ..Recently, the human GLI3 gene has been proposed to be a candidate gene for GCPS...
  94. ncbi The role of Alx-4 in the establishment of anteroposterior polarity during vertebrate limb development
    M Takahashi
    The Salk Institute for Biological Studies, Gene Expression Laboratory, La Jolla, CA 92037 1099, USA
    Development 125:4417-25. 1998
    ..Analysis of polydactylous mutants indicate that the interaction between Alx-4 and Shh is independent of Gli3, a negative regulator of Shh in the limb...
  95. ncbi Gli3 is required for Emx gene expression during dorsal telencephalon development
    T Theil
    Institute for Molecular Biology, Hannover Medical School, Carl Neuberg Strasse 1, D 30625 Hannover, Germany thomas
    Development 126:3561-71. 1999
    ..Here, we report on forebrain development in the extra toes (Xt(J)) mouse mutant which carries a null mutation of the Gli3 gene...
  96. ncbi Specification of ventral neuron types is mediated by an antagonistic interaction between Shh and Gli3
    Y Litingtung
    Department of Cell Biology, Vanderbilt University Medical Center, B2120 MCN, Nashville, Tennessee 37232, USA
    Nat Neurosci 3:979-85. 2000
    ..Here we show that Gli3 represses ventral fates in a dose-dependent manner...
  97. ncbi Pallister-Hall syndrome phenotype in mice mutant for Gli3
    Jens Böse
    Institute for Animal Developmental and Molecular Biology, Heinrich Heine University, Universitatsstrasse 1, 40225 Dusseldorf, Germany
    Hum Mol Genet 11:1129-35. 2002
    Mutations in the GLI3 gene have been identified in several human malformation syndromes...
  98. ncbi Embryonic retinoic acid synthesis is required for forelimb growth and anteroposterior patterning in the mouse
    Karen Niederreither
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM ULP College de France, BP 10142, 67404 Illkirch Cedex, CU de Strasbourg, France
    Development 129:3563-74. 2002
    ..We suggest that RA signaling cooperates with a posteriorly restricted factor such as dHand, to generate a functional zone of polarizing activity (ZPA)...
  99. ncbi Shh and Gli3 are dispensable for limb skeleton formation but regulate digit number and identity
    Ying Litingtung
    Vanderbilt University Medical Center, Department of Cell and Developmental Biology, Nashville, Tennessee 37232, USA
    Nature 418:979-83. 2002
    ..b>Gli3 (the gene implicated in human Greig cephalopolysyndactyly syndrome) is proposed to negatively regulate Shh by ..
  100. ncbi Differential requirement for Gli2 and Gli3 in ventral neural cell fate specification
    Jun Motoyama
    Molecular Neuropathology Group, Brain Research Institute, RIKEN, 2 1 Hirosawa, Wako, Saitama 351 0198, Japan
    Dev Biol 259:150-61. 2003
    ..Here, we show that the transcription factors Gli2 and Gli3, mediators of Shh signaling, are required for the development of the ventral cell fates but make distinct ..
  101. ncbi Direct interaction with Hoxd proteins reverses Gli3-repressor function to promote digit formation downstream of Shh
    Yuting Chen
    Laboratory of Pathology, Center for Cancer Research, NCI, NIH, Bethesda, MD 20892, USA
    Development 131:2339-47. 2004
    ..Shh regulates digit formation largely by preventing cleavage of the Gli3 transcription factor to a repressor form that shuts off expression of Shh target genes...

Research Grants5

  1. Excess TGF beta in neonatal lung injury and repair
    David Warburton; Fiscal Year: 2004
    ..abstract_text> ..
  2. Lung epithelial and vascular morphogenesis
    David Warburton; Fiscal Year: 2006
    ..abstract_text> ..
  3. Eda/Edar Regulation of Embryonic SMG Development
    Tina Jaskoll; Fiscal Year: 2007
    ....
  4. AUTOCRINE/PARACRINE GROWTH FACTORS & LUNG MORPHOGENESIS
    David Warburton; Fiscal Year: 2009
    ..Aim 4: To determine the role of tyrosine phosphorylation in protein-protein interaction and supra-molecular assembly of mSPRY2 with SHP2 and FRS2. Aim 5: To determine the functional importance of Shp2 in lung morphogenesis in vivo. ..
  5. DEVELOPMENTAL TYPE II PNEUMOCYTE PROTEIN PHOSPHORYLATION
    David Warburton; Fiscal Year: 2010
    ..Inosine may function as a low molecular weight therapeutic to ameliorate lung injury, protect lung progenitor cells and hence to promote recovery of the alveolar epithelium from acute injury in both adult and neonate. ..