Nf1

Summary

Gene Symbol: Nf1
Description: neurofibromin 1
Alias: AW494271, Dsk9, E030030H24Rik, Mhdadsk9, Nf-1, neurofibromin, neurofibromatosis 1, neurofibromatosis-related protein NF-1
Species: mouse

Top Publications

  1. ncbi Mechanism for the learning deficits in a mouse model of neurofibromatosis type 1
    Rui M Costa
    Departments of Neurobiology, Psychiatry and Psychology, BRI, University of California at Los Angeles, Los Angeles, California 90095 1761, USA
    Nature 415:526-30. 2002
  2. ncbi The neurofibromatosis 1 gene product neurofibromin regulates pituitary adenylate cyclase-activating polypeptide-mediated signaling in astrocytes
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 23:8949-54. 2003
  3. ncbi Neurofibromin binds to caveolin-1 and regulates ras, FAK, and Akt
    Madanamohan Boyanapalli
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA
    Biochem Biophys Res Commun 340:1200-8. 2006
  4. ncbi Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri, USA
    Cancer Res 65:2755-60. 2005
  5. doi A role for Dimethylarginine Dimethylaminohydrolase 1 (DDAH1) in mammalian development
    Ross A Breckenridge
    Centre for Clinical Pharmacology, University College London, BHF Laboratories, London, UK
    Int J Dev Biol 54:215-20. 2010
  6. pmc Mouse tumor model for neurofibromatosis type 1
    K S Vogel
    Center for Developmental Biology and Department of Pathology, University of Texas Southwestern Medical Center, 6000 Harry Hines Blvd, Dallas, TX 75235 9133, USA
    Science 286:2176-9. 1999
  7. pmc Neurofibromatosis-1 regulates neuroglial progenitor proliferation and glial differentiation in a brain region-specific manner
    Da Yong Lee
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Genes Dev 24:2317-29. 2010
  8. pmc The loss of Nf1 transiently promotes self-renewal but not tumorigenesis by neural crest stem cells
    Nancy M Joseph
    Center for Stem Cell Biology, Howard Hughes Medical Institute, Life Sciences Institute, University of Michigan, Ann Arbor, MI 48109 2216, USA
    Cancer Cell 13:129-40. 2008
  9. pmc Neurofibromatosis-1 regulates mTOR-mediated astrocyte growth and glioma formation in a TSC/Rheb-independent manner
    Sutapa Banerjee
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 108:15996-6001. 2011
  10. pmc Neurofibromatosis-1 heterozygosity impairs CNS neuronal morphology in a cAMP/PKA/ROCK-dependent manner
    Jacquelyn A Brown
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Mol Cell Neurosci 49:13-22. 2012

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Scientific Experts

Detail Information

Publications142 found, 100 shown here

  1. ncbi Mechanism for the learning deficits in a mouse model of neurofibromatosis type 1
    Rui M Costa
    Departments of Neurobiology, Psychiatry and Psychology, BRI, University of California at Los Angeles, Los Angeles, California 90095 1761, USA
    Nature 415:526-30. 2002
    ..2). Although neurofibromin has several known properties and functions, including Ras GTPase-activating protein activity, adenylyl cyclase ..
  2. ncbi The neurofibromatosis 1 gene product neurofibromin regulates pituitary adenylate cyclase-activating polypeptide-mediated signaling in astrocytes
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 23:8949-54. 2003
    Individuals with the neurofibromatosis 1 (NF1)-inherited tumor predisposition syndrome develop low-grade astrocytomas...
  3. ncbi Neurofibromin binds to caveolin-1 and regulates ras, FAK, and Akt
    Madanamohan Boyanapalli
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA
    Biochem Biophys Res Commun 340:1200-8. 2006
    b>Neurofibromin (Nf1) is an approximately 280 kDa protein having tumor suppressor function, presumably by virtue of its GTPase activating domain, but little is known regarding molecular aspects of its effector pathways...
  4. ncbi Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri, USA
    Cancer Res 65:2755-60. 2005
    Individuals with the tumor predisposition syndrome, neurofibromatosis 1 (NF1), are prone to development of nervous system tumors, including neurofibromas and pilocytic astrocytomas...
  5. doi A role for Dimethylarginine Dimethylaminohydrolase 1 (DDAH1) in mammalian development
    Ross A Breckenridge
    Centre for Clinical Pharmacology, University College London, BHF Laboratories, London, UK
    Int J Dev Biol 54:215-20. 2010
    ..These expression patterns implicate DDAH1 and DDAH2 in embryonic development, possibly through specific effects on nitric oxide pathways...
  6. pmc Mouse tumor model for neurofibromatosis type 1
    K S Vogel
    Center for Developmental Biology and Department of Pathology, University of Texas Southwestern Medical Center, 6000 Harry Hines Blvd, Dallas, TX 75235 9133, USA
    Science 286:2176-9. 1999
    Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors of neural crest origin...
  7. pmc Neurofibromatosis-1 regulates neuroglial progenitor proliferation and glial differentiation in a brain region-specific manner
    Da Yong Lee
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Genes Dev 24:2317-29. 2010
    ..In this study, we used neurofibromatosis-1 (NF1) as a model system to elucidate a novel molecular mechanism underlying brain region-specific NSC functional ..
  8. pmc The loss of Nf1 transiently promotes self-renewal but not tumorigenesis by neural crest stem cells
    Nancy M Joseph
    Center for Stem Cell Biology, Howard Hughes Medical Institute, Life Sciences Institute, University of Michigan, Ann Arbor, MI 48109 2216, USA
    Cancer Cell 13:129-40. 2008
    Neurofibromatosis is caused by the loss of neurofibromin (Nf1), leading to peripheral nervous system (PNS) tumors, including neurofibromas and malignant peripheral nerve sheath tumors (MPNSTs)...
  9. pmc Neurofibromatosis-1 regulates mTOR-mediated astrocyte growth and glioma formation in a TSC/Rheb-independent manner
    Sutapa Banerjee
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 108:15996-6001. 2011
    ..This shared dependence on mTOR suggests that PTEN and NF1 (neurofibromin) glial growth regulation requires TSC/Rheb (Ras homolog enriched in brain) control of mTOR function...
  10. pmc Neurofibromatosis-1 heterozygosity impairs CNS neuronal morphology in a cAMP/PKA/ROCK-dependent manner
    Jacquelyn A Brown
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Mol Cell Neurosci 49:13-22. 2012
    ..These clinical features result from reduced NF1 protein (neurofibromin) expression in NF1+/- (NF1 heterozygosity) brain neurons...
  11. ncbi The interferon consensus sequence-binding protein activates transcription of the gene encoding neurofibromin 1
    Chunliu Zhu
    Feinberg School of Medicine and the Robert H Lurie Comprehensive Cancer Center, Northwestern University and Chicago Lakeside Veterans Affairs Hospital, Chicago, Illinois 60611, USA
    J Biol Chem 279:50874-85. 2004
    ..In these studies, we identify the gene encoding neurofibromin 1 (Nf1) as an ICSBP target gene, by chromatin immunoprecipitation...
  12. doi Neurofibromatosis type 1 (NF1) tumor suppressor, neurofibromin, regulates the neuronal differentiation of PC12 cells via its associating protein, CRMP-2
    Siriporn Patrakitkomjorn
    Department of Tumor Genetics and Biology, Graduate School of Medical Sciences, Kumamoto University School of Medicine, Kumamoto, Japan
    J Biol Chem 283:9399-413. 2008
    Neurofibromatosis type 1 (NF1) tumor suppressor gene product, neurofibromin, functions in part as a Ras-GAP, a negative regulator of Ras...
  13. ncbi Restored plasticity in a mouse model of neurofibromatosis type 1 via inhibition of hyperactive ERK and CREB
    Clare Guilding
    Institute of Biomedical and Life Sciences, West Medical Building, University of Glasgow, Glasgow G12 8QQ, UK
    Eur J Neurosci 25:99-105. 2007
    Patients with neurofibromatosis type 1 (NF1), resulting from neurofibromin gene mutations, frequently suffer from deficits in learning and spatial memory...
  14. pmc Targeting neovascular pericytes in neurofibromatosis type 1
    Ugur Ozerdem
    Vascular Biology Division, La Jolla Institute for Molecular Medicine, 4570 Executive Drive, Suite 100, San Diego, California, 92121, USA
    Angiogenesis 7:307-11. 2004
    Apart from tumor-driven neovascularization, a less-appreciated consequence of neurofibromatosis type 1 (NF1) is the hyperproliferation of vascular mural cells (pericytes)...
  15. ncbi Spatiotemporal differences in CXCL12 expression and cyclic AMP underlie the unique pattern of optic glioma growth in neurofibromatosis type 1
    Nicole M Warrington
    Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Cancer Res 67:8588-95. 2007
    Astrocytoma (glioma) formation in neurofibromatosis type 1 (NF1) occurs preferentially along the optic pathway during the first decade of life. The molecular basis for this unique pattern of gliomagenesis is unknown...
  16. doi Motor deficits in neurofibromatosis type 1 mice: the role of the cerebellum
    T van der Vaart
    Department of Neuroscience, Erasmus Medical Center, Rotterdam, The Netherlands
    Genes Brain Behav 10:404-9. 2011
    ..Cerebellar Purkinje cells, the sole output neurons of the cerebellar cortex, are GABAergic neurons and express neurofibromin at high levels, suggesting an important role for the cerebellum in the observed motor deficits in NF1...
  17. pmc c-Fms signaling mediates neurofibromatosis Type-1 osteoclast gain-in-functions
    Yongzheng He
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, United States of America
    PLoS ONE 7:e46900. 2012
    ..including osteoporosis and osteopenia occur frequently in both pediatric and adult neurofibromatosis type 1 (NF1) patients...
  18. pmc Region-specific astrogliosis in brains of mice heterozygous for mutations in the neurofibromatosis type 1 (Nf1) tumor suppressor
    T A Rizvi
    Department of Cell Biology, Neurobiology and Anatomy, University of Cincinnati College of Medicine, 231 Bethesda Avenue, Cincinnati, OH, 45267 0521, USA
    Brain Res 816:111-23. 1999
    ..L. Nordlund, T.A. Rizvi, C.I. Brannan, N. Ratner, Neurofibromin expression and astrogliosis in neurofibromatosis (type 1) brains, J. Neuropathol. Exp...
  19. ncbi Glioma formation in neurofibromatosis 1 reflects preferential activation of K-RAS in astrocytes
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Cancer Res 65:236-45. 2005
    Children with the tumor predisposition syndrome, neurofibromatosis 1 (NF1), develop optic pathway gliomas...
  20. pmc Detection and measurement of neurofibromatosis-1 mouse optic glioma in vivo
    Debasish Banerjee
    Department of Radiology, Washington University School of Medicine, St Louis, MO 63110, USA
    Neuroimage 35:1434-7. 2007
    ..We present a rapid and reliable method to detect optic glioma (OPG) in a mouse neurofibromatosis-1 model (Nf1(flox/mut)GFAP-Cre mice) in vivo using Manganese-Enhanced Magnetic Resonance Imaging (MEMRI)...
  21. pmc Defective cAMP generation underlies the sensitivity of CNS neurons to neurofibromatosis-1 heterozygosity
    Jacquelyn A Brown
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 30:5579-89. 2010
    ..Nf1+/- CNS neuronal phenotypes do not reflect Ras pathway hyperactivation, but rather result from impaired neurofibromin-mediated cAMP generation...
  22. pmc Cyclic AMP suppression is sufficient to induce gliomagenesis in a mouse model of neurofibromatosis-1
    Nicole M Warrington
    Department of Pediatrics, Molecular Imaging Center, Mallinckrodt Institute of Radiology, Developmental Biology, and Anatomy and Neurobiology, Washington University School of Medicine and St Louis Children s Hospital, St Louis, Missouri 63110, USA
    Cancer Res 70:5717-27. 2010
    ..Previous studies from our laboratories have used neurofibromatosis-1 (NF1) genetically engineered mouse (GEM) models to understand the spatial restriction of glioma formation to the optic ..
  23. pmc Astrocyte-specific inactivation of the neurofibromatosis 1 gene (NF1) is insufficient for astrocytoma formation
    Michaela Livia Bajenaru
    Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Mol Cell Biol 22:5100-13. 2002
    Individuals with the neurofibromatosis 1 (NF1) inherited tumor syndrome develop low-grade gliomas (astrocytomas) at an increased frequency, suggesting that the NF1 gene is a critical growth regulator for astrocytes...
  24. ncbi MRI abnormalities in neurofibromatosis type 1 (NF1): a study of men and mice
    T Rosenbaum
    Department of Neuropediatrics, Children s Hospital, Heinrich Heine University, Dusseldorf, Germany
    Brain Dev 21:268-73. 1999
    ..Additionally, brain MR imaging of Nf1 knockout mice was performed to find out if similar abnormalities are present in this animal model...
  25. doi Models of tibial fracture healing in normal and Nf1-deficient mice
    Aaron Schindeler
    Department of Orthopaedic Research and Biotechnology, Reasearch Building, Locked Bag 4001, The Children s Hospital at Westmead, Sydney, NSW 2145, Australia
    J Orthop Res 26:1053-60. 2008
    ..These tibial fracture models were applied to both wild-type and Nf1-deficient (Nf1+/-) mice...
  26. pmc Identification of the neurofibromatosis type 1 gene product
    D H Gutmann
    Department of Internal Medicine, University of Michigan, Ann Arbor 48109 0650
    Proc Natl Acad Sci U S A 88:9658-62. 1991
    The gene for neurofibromatosis type 1 (NF1) was recently identified by positional cloning. The complete cDNA encodes a polypeptide of 2818 amino acids...
  27. ncbi Sequence homology shared by neurofibromatosis type-1 gene and IRA-1 and IRA-2 negative regulators of the RAS cyclic AMP pathway
    A M Buchberg
    ABL Basic Research Program, NCI Frederick Cancer Research and Development Center, Maryland 21702
    Nature 347:291-4. 1990
    ..These findings provide important new insights into the possible function of the NF-1 gene...
  28. ncbi Neurofibromatosis 1 (NF1) mRNAs expressed in the central nervous system are differentially spliced in the 5' part of the gene
    G Danglot
    Cytogenetique et Genetique Oncologiques, CNRS URA 1158, Institut Gustave Roussy, Villejuif, France
    Hum Mol Genet 4:915-20. 1995
    The neurofibromatosis 1 gene seems to play essential roles at several different stages of life...
  29. ncbi Mouse neurofibromatosis type 1 cDNA sequence reveals high degree of conservation of both coding and non-coding mRNA segments
    A Bernards
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Boston 02129
    Hum Mol Genet 2:645-50. 1993
    ..The sequence predicts a 2841 amino acid protein that is more than 98% identical to human neurofibromin. All but 9 of the 45 amino acid differences between mouse and human neurofibromin occur in the N-terminal half ..
  30. ncbi The neurofibromatosis type 1 (NF1) tumor suppressor gene and myeloid leukemia
    D A Largaespada
    Mammalian Genetics Laboratory, NCI Frederick Cancer Research and Development Center, MD 21702, USA
    Curr Top Microbiol Immunol 211:233-9. 1996
  31. ncbi Predetermined chromosomal deletion encompassing the Nf-1 gene
    T Schlake
    Department of Developmental Immunology, Max Planck Institute for Immunobiology, Stuebeweg 51, D 79108 Freiburg, Germany
    Oncogene 18:6078-82. 1999
    ..This strategy may be developed into a scheme by which large chromosomal regions with precisely defined end points may be excised from mammalian cells and reintroduced after suitable in vitro modification...
  32. ncbi Neurofibromin, the neurofibromatosis type 1 Ras-GAP, is required for appropriate P0 expression and myelination
    T Rosenbaum
    Department of Cell Biology, Neurobiology, and Anatomy, University of Cincinnati Medical School, Ohio 45267 0521, USA
    Ann N Y Acad Sci 883:203-14. 1999
    The neurofibromatosis type 1 (NF1) gene product, neurofibromin, regulates activation of the Ras intracellular signaling pathway in Schwann cells...
  33. ncbi Regulation of the murine Ddelta2 promoter by upstream stimulatory factor 1, Runx1, and c-Myb
    Juan Carabana
    Department of Immunology, Duke University Medical Center, Durham, NC 27710, USA
    J Immunol 174:4144-52. 2005
    ..EMSAs reveal that upstream stimulatory factor 1, Runx1, c-Myb, lymphoid enhancer binding factor 1, NF1, and E47 all interact with this promoter in vitro...
  34. ncbi Cerebrospinal fluid proteomic analysis reveals dysregulation of methionine aminopeptidase-2 expression in human and mouse neurofibromatosis 1-associated glioma
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Cancer Res 65:9843-50. 2005
    Individuals affected with the neurofibromatosis 1 (NF1) tumor predisposition syndrome are prone to the development of multiple nervous system tumors, including optic pathway gliomas (OPG)...
  35. pmc Microarray analyses reveal regional astrocyte heterogeneity with implications for neurofibromatosis type 1 (NF1)-regulated glial proliferation
    Tu Hsueh Yeh
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Glia 57:1239-49. 2009
    ..Interestingly, neurofibromatosis type 1 (NF1) gene expression was decreased at both the RNA and protein levels in neocortical astroglia relative to astroglia ..
  36. ncbi Tumour predisposition in mice heterozygous for a targeted mutation in Nf1
    T Jacks
    Center for Cancer Research, Massachusetts Institute of Technology, Cambridge 02139
    Nat Genet 7:353-61. 1994
    Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue...
  37. ncbi Targeted disruption of the neurofibromatosis type-1 gene leads to developmental abnormalities in heart and various neural crest-derived tissues
    C I Brannan
    Mammalian Genetics Laboratory, Frederick Cancer Research and Development Center, Maryland 21702 1201
    Genes Dev 8:1019-29. 1994
    ..These results identify new roles for NF1 in development and indicate that some of the abnormal growth phenomena observed in NF1 patients can be recapitulated in neurofibromin-deficient mice.
  38. ncbi Nf1 deficiency causes Ras-mediated granulocyte/macrophage colony stimulating factor hypersensitivity and chronic myeloid leukaemia
    D A Largaespada
    Mammalian Genetics Laboratory, NCI Frederick Cancer Research and Development Center, Maryland 21702, USA
    Nat Genet 12:137-43. 1996
    ..chronic myelogenous leukaemia (JCML), while other patients with JCML show loss of the neurofibromatosis type 1 (NF1) gene, a Ras GTPase activating protein...
  39. pmc The Nf1 tumor suppressor regulates mouse skin wound healing, fibroblast proliferation, and collagen deposited by fibroblasts
    R P Atit
    Division of Biology, Children s Hospital Research Foundation, Cincinnati, Ohio, USA
    J Invest Dermatol 112:835-42. 1999
    ..Trauma has been proposed to trigger neurofibroma formation. To test if loss of the neurofibromatosis type 1 gene (Nf1) compromises fibroblast function in vivo following trauma, skin wounding was performed in Nf1 knockout mice...
  40. ncbi Mouse models of tumor development in neurofibromatosis type 1
    K Cichowski
    Department of Biology and Center for Cancer Research and Howard Hughes Medical Institute, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Science 286:2172-6. 1999
    Neurofibromatosis type 1 (NF1) is a prevalent familial cancer syndrome resulting from germ line mutations in the NF1 tumor suppressor gene...
  41. pmc Genetic and biochemical evidence that haploinsufficiency of the Nf1 tumor suppressor gene modulates melanocyte and mast cell fates in vivo
    D A Ingram
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    J Exp Med 191:181-8. 2000
    ..Recently neurofibromin, the protein encoded by NF1, was shown to negatively regulate Ras activity in Nf1-/- murine myeloid ..
  42. ncbi Nf1 and Gmcsf interact in myeloid leukemogenesis
    R A Birnbaum
    Department of Pediatrics, University of California, San Francisco 94143 0519, USA
    Mol Cell 5:189-95. 2000
    The NF1 tumor suppressor gene encodes neurofibromin, a GTPase-activating protein (GAP) for p21ras (Ras). Children with NF1 are predisposed to juvenile myelomonocytic leukemia (JMML)...
  43. pmc The neurofibromatosis type 1 (Nf1) tumor suppressor is a modifier of carcinogen-induced pigmentation and papilloma formation in C57BL/6 mice
    R P Atit
    Division of Molecular and Developmental Biology, Children s Hospital Research Foundation, Cincinnati, OH, USA
    J Invest Dermatol 114:1093-100. 2000
    ..in codon 61 of H-ras, demonstrating strong cooperation between the Nf1 GTPase activating protein for Ras, neurofibromin, and Ras-GTP...
  44. ncbi Nf1;Trp53 mutant mice develop glioblastoma with evidence of strain-specific effects
    K M Reilly
    Department of Biology and Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA
    Nat Genet 26:109-13. 2000
    ..We present here a mouse model of astrocytomas involving mutation of two tumour-suppressor genes, Nf1 and Trp53...
  45. ncbi Neurofibromatosis 1 (NF1) heterozygosity results in a cell-autonomous growth advantage for astrocytes
    M L Bajenaru
    Department of Neurology, Washington University School of Medicine, 860 S Euclid Avenue, St Louis, MO 63110, USA
    Glia 33:314-23. 2001
    Individuals with neurofibromatosis 1 (NF1) develop low-grade astrocytomas at an increased frequency...
  46. pmc Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain
    Y Zhu
    Center for Developmental Biology, University of Texas Southwestern Medical Center, Dallas, Texas 75390 9133, USA
    Genes Dev 15:859-76. 2001
    Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities...
  47. ncbi Analysis of melanocyte precursors in Nf1 mutants reveals that MGF/KIT signaling promotes directed cell migration independent of its function in cell survival
    B Wehrle-Haller
    Institute of Neuroscience, University of Oregon, Eugene, Oregon 97403 1254, USA
    Dev Biol 232:471-83. 2001
    ..from chemoattractive or motogenic functions of this ligand using mice that carry a targeted mutation at the Neurofibromin (Nf1) locus and consequently lack RAS-GAP function...
  48. ncbi Defective proliferative responses in B lymphocytes and thymocytes that lack neurofibromin
    Tae Jin Kim
    Cancer Center, Massachusetts General Hospital, Building 149, 13th Street, Charlestown Navy Yard, Boston, MA 02129, USA
    Mol Immunol 38:701-8. 2002
    ..Lymphocyte development was largely unimpaired in the absence of neurofibromin. However antigen-receptor induced proliferation was defective in neurofibromin deficient peripheral B cells ..
  49. pmc Neurofibromas in NF1: Schwann cell origin and role of tumor environment
    Yuan Zhu
    Center for Developmental Biology, Department of Pathology, University of Texas Southwestern Medical Center, 6000 Harry Hines Boulevard, Dallas, TX 75390 9133, USA
    Science 296:920-2. 2002
    Neurofibromatosis type 1 (NF1) is one of the most prevalent dominantly inherited genetic diseases of the nervous system...
  50. ncbi Lymphoproliferative defects in mice lacking the expression of neurofibromin: functional and biochemical consequences of Nf1 deficiency in T-cell development and function
    David A Ingram
    Section of Neonatal Perinatal Medicine, Department of Pediatrics, Herman B Wells Center for Pediatric Research, Indianapolis, IN 46202, USA
    Blood 100:3656-62. 2002
    ..In this study we demonstrate that neurofibromin, the protein encoded by the NF1 tumor suppressor gene functions as a GAP for Ras in T cells...
  51. ncbi Neurofibromatosis 1: closing the GAP between mice and men
    Biplab Dasgupta
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Curr Opin Genet Dev 13:20-7. 2003
    b>Neurofibromatosis 1 (NF1) is a common genetic condition in which affected individuals are prone to the development of benign and malignant tumors...
  52. ncbi Aberrant growth and differentiation of oligodendrocyte progenitors in neurofibromatosis type 1 mutants
    Michael R Bennett
    Department of Cell Biology, Neurobiology, and Anatomy, University of Cincinnati College of Medicine, Cincinnati, Ohio 45267 0521, USA
    J Neurosci 23:7207-17. 2003
    ..The data reveal a key role for neurofibromin and Ras signaling in the maintenance of CNS progenitor cell pools and also suggest a potential role for ..
  53. pmc Neurofibromin-deficient Schwann cells secrete a potent migratory stimulus for Nf1+/- mast cells
    Feng Chun Yang
    Department of Pediatrics, Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    J Clin Invest 112:1851-61. 2003
    The NF1 tumor suppressor gene encodes a GTPase-activating protein called neurofibromin that negatively regulates Ras signaling. Mutations in NF1 cause neurofibromatosis type 1 (NF1)...
  54. ncbi Optic nerve glioma in mice requires astrocyte Nf1 gene inactivation and Nf1 brain heterozygosity
    M Livia Bajenaru
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Cancer Res 63:8573-7. 2003
    Whereas biallelic neurofibromatosis 1 (NF1) inactivation is observed in NF1-associated gliomas, astrocyte-restricted Nf1 conditional knockout mice do not develop gliomas...
  55. ncbi Somatic inactivation of Nf1 in hematopoietic cells results in a progressive myeloproliferative disorder
    Doan T Le
    Department of Pediatrics, University of California, San Francisco, 513 Parnassus Ave, HSE 302, San Francisco, CA 94143, USA
    Blood 103:4243-50. 2004
    The NF1 tumor suppressor gene encodes a guanosine triphosphotase (GTPase)-activating protein that negatively regulates Ras signaling and is inactivated in a subset of juvenile myelomonocytic leukemias (JMMLs)...
  56. ncbi NF1 tumor suppressor protein and mRNA in skeletal tissues of developing and adult normal mouse and NF1-deficient embryos
    Tommi Kuorilehto
    Department of Anatomy and Cell Biology, University of Oulu, Oulu, Finland
    J Bone Miner Res 19:983-9. 2004
    b>NF1 is a heritable disease with multiple osseous lesions. The expression of the NF1 gene was studied in embryonic and adult rodent skeleton and in NF1-deficient embryos...
  57. ncbi Natural history of neurofibromatosis 1-associated optic nerve glioma in mice
    M Livia Bajenaru
    Department of Neurology, Washington University School of Medicine, 660 S Euclid Avenue, St Louis, MO 63110, USA
    Ann Neurol 57:119-27. 2005
    Children affected with the inherited tumor predisposition syndrome, neurofibromatosis 1 (NF1), are prone to the development of low-grade astrocytic optic pathway tumors (optic pathway glioma [OPG])...
  58. ncbi Neurofibromin and its inactivation of Ras are prerequisites for osteoblast functioning
    X Yu
    Indiana University School of Medicine, Indianapolis, IN 46202, USA
    Bone 36:793-802. 2005
    ..Inactivation of neurofibromin results in deregulation of Ras signal transduction. Little is known of bone biology in humans with NF1...
  59. pmc The NF1 tumor suppressor critically regulates TSC2 and mTOR
    Cory M Johannessen
    Genetics Division, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, 458c NRB, 77 Louis Pasteur Avenue, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 102:8573-8. 2005
    ..The NF1-encoded protein, neurofibromin, functions as a Ras-GTPase activating protein (RasGAP)...
  60. ncbi Sensory neurons from Nf1 haploinsufficient mice exhibit increased excitability
    Yue Wang
    Departments of Pharmacology and Toxicology, Indiana University School of Medicine, Indianapolis, 46202, USA
    J Neurophysiol 94:3670-6. 2005
    ..NF1 results from a heterozygous mutation of the NF1 gene, leading to decreased levels of neurofibromin, the protein product of the NF1 gene...
  61. pmc Early inactivation of p53 tumor suppressor gene cooperating with NF1 loss induces malignant astrocytoma
    Yuan Zhu
    Center for Developmental Biology and Kent Waldrep Foundation Center for Basic Research on Nerve Growth and Regeneration, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Cancer Cell 8:119-30. 2005
    ..We have generated mouse strains that lack p53 and harbor a conditional allele of the NF1 tumor suppressor that negatively regulates Ras signaling...
  62. ncbi The HMG-CoA reductase inhibitor lovastatin reverses the learning and attention deficits in a mouse model of neurofibromatosis type 1
    Weidong Li
    Department of Neurobiology, University of California, Los Angeles, Los Angeles, California 90095, USA
    Curr Biol 15:1961-7. 2005
    Neurofibromatosis Type 1 (NF1) is a common neurological disorder caused by mutations in the gene encoding Neurofibromin, a p21Ras GTPase Activating Protein (GAP). Importantly, NF1 causes learning disabilities and attention deficits...
  63. ncbi Nf1 haploinsufficiency augments angiogenesis
    M Wu
    Department of Pediatrics, Division of Neurology, University of Florida, Gainesville, FL 32610, USA
    Oncogene 25:2297-303. 2006
    Mutations in the NF1 tumor-suppressor gene underlie neurofibromatosis type 1 (NF1), in which patients are predisposed to certain tumors such as neurofibromas and may associate with vascular disorder...
  64. ncbi Stimulus-evoked release of neuropeptides is enhanced in sensory neurons from mice with a heterozygous mutation of the Nf1 gene
    C M Hingtgen
    Department of Neurology, Stark Neurosciences Research Institute, Indiana University School of Medicine, 950 West Walnut Street, R2 466, Indianapolis, 46202, USA
    Neuroscience 137:637-45. 2006
    ..Neurofibrinomin, the protein product of the NF1 gene (neurofibromin gene (human)), is a guanosine triphosphate activating protein for p21(ras)...
  65. pmc Inactivation of NF1 in CNS causes increased glial progenitor proliferation and optic glioma formation
    Yuan Zhu
    Center for Developmental Biology and Kent Waldrep Foundation Center for Basic Research on Nerve Growth and Regeneration, University of Texas Southwestern Medical Center, Dallas, TX 75390 9133, USA
    Development 132:5577-88. 2005
    The gene responsible for neurofibromatosis type 1 (NF1) encodes a tumor suppressor that functions as a negative regulator of the Ras proto-oncogene...
  66. ncbi Neurofibromin plays a critical role in modulating osteoblast differentiation of mesenchymal stem/progenitor cells
    Xiaohua Wu
    Department of Pediatrics, Herman B Wells Center for Pediatric Research and Indiana University School of Medicine, Cancer Research Institute, Indianapolis 46202, USA
    Hum Mol Genet 15:2837-45. 2006
    ..Taken together, our results suggest that neurofibromin plays a crucial role in modulating MSPC differentiation into osteoblasts, and the defect in osteoblast ..
  67. pmc Hyperactivation of p21ras and PI3K cooperate to alter murine and human neurofibromatosis type 1-haploinsufficient osteoclast functions
    Feng Chun Yang
    Department of Pediatrics, Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    J Clin Invest 116:2880-91. 2006
    Individuals with neurofibromatosis type 1 (NF1) have a high incidence of osteoporosis and osteopenia. However, understanding of the cellular and molecular basis of these sequelae is incomplete...
  68. pmc ATF4 mediation of NF1 functions in osteoblast reveals a nutritional basis for congenital skeletal dysplasiae
    Florent Elefteriou
    Department of Molecular and Human Genetics, Bone Disease Program of Texas, Baylor College of Medicine, One Baylor Plaza, Houston, Texas 77030, USA
    Cell Metab 4:441-51. 2006
    ..contrast, RSK2 activity, ATF4-dependent collagen synthesis, and bone formation are increased in mice lacking neurofibromin in osteoblasts (Nf1(ob)(-/-) mice)...
  69. ncbi A mild mutator phenotype arises in a mouse model for malignancies associated with neurofibromatosis type 1
    Rene Garza
    Department of Cellular and Structural Biology, The University of Texas, Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229 3900, USA
    Mutat Res 615:98-110. 2007
    ..Although most tumors that arise in humans with neurofibromatosis type 1 (NF1) are benign, these individuals are at increased risk for malignant peripheral nerve sheath tumors (MPNST)...
  70. ncbi Nf1 expression is dependent on strain background: implications for tumor suppressor haploinsufficiency studies
    Jessica J Hawes
    Mouse Cancer Genetics Program, National Cancer Institute Frederick, West 7th Street at Fort Detrick, P O Box B, Building 560, Rm 31 20, Frederick, MD 21702, USA
    Neurogenetics 8:121-30. 2007
    Neurofibromatosis type 1 (NF1) is the most common cancer predisposition syndrome affecting the nervous system, with elevated risk for both astrocytoma and peripheral nerve sheath tumors...
  71. ncbi Multiple roles for neurofibromin in skeletal development and growth
    Mateusz Kolanczyk
    FG Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany
    Hum Mol Genet 16:874-86. 2007
    ..To investigate the role of the neurofibromin in skeletal development, we crossed Nf1flox mice with Prx1Cre mice to inactivate Nf1 in undifferentiated ..
  72. ncbi Rac1 mediates the osteoclast gains-in-function induced by haploinsufficiency of Nf1
    Jincheng Yan
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    Hum Mol Genet 17:936-48. 2008
    Neurofibromatosis type I (NF1) is a congenital disorder resulting from loss-of-function of the tumor suppressor gene, NF1, a GTPase-activating protein for p21ras...
  73. ncbi Neurofibromin as a regulator of melanocyte development and differentiation
    Ganesh Diwakar
    Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA
    J Cell Sci 121:167-77. 2008
    ..characteristic pigmentary lesions associated with loss of a single allele of NF1, encoding the 260 kDa protein neurofibromin. To understand the basis for these pigmentary problems, the properties of melanocytes haploinsufficient for ..
  74. doi Preclinical cancer therapy in a mouse model of neurofibromatosis-1 optic glioma
    Balazs Hegedus
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Cancer Res 68:1520-8. 2008
    ..Because neurofibromin negatively regulates mammalian target of rapamycin (mTOR) signaling, we showed that pharmacologic mTOR ..
  75. doi Neurofibromatosis-1 regulates neuronal and glial cell differentiation from neuroglial progenitors in vivo by both cAMP- and Ras-dependent mechanisms
    Balazs Hegedus
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Cell Stem Cell 1:443-57. 2007
    ..type 1 (NF1) develop abnormalities of both neuronal and glial cell lineages, suggesting that the NF1 protein neurofibromin is an essential regulator of neuroglial progenitor function...
  76. pmc Pten haploinsufficiency accelerates formation of high-grade astrocytomas
    Chang Hyuk Kwon
    Department of Developmental Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390 9133, USA
    Cancer Res 68:3286-94. 2008
    We previously reported that central nervous system (CNS) inactivation of Nf1 and p53 tumor suppressor genes in mice results in the development of low-grade to high-grade progressive astrocytomas...
  77. pmc Neurofibromin regulates somatic growth through the hypothalamic-pituitary axis
    Balazs Hegedus
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Hum Mol Genet 17:2956-66. 2008
    ..We further demonstrate that the small anterior pituitary size reflects loss of neurofibromin expression in the hypothalamus, leading to reduced growth hormone releasing hormone, pituitary growth hormone (..
  78. pmc Nf1-dependent tumors require a microenvironment containing Nf1+/-- and c-kit-dependent bone marrow
    Feng Chun Yang
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    Cell 135:437-48. 2008
    ..Germline mutations in the NF1 tumor suppressor gene cause neurofibromatosis type 1 (NF1), a common genetic disorder characterized by complex ..
  79. pmc Neurofibromin regulation of ERK signaling modulates GABA release and learning
    Yijun Cui
    Department of Neurobiology, Brain Research Institute, University of California, Los Angeles, Los Angeles, CA 90095, USA
    Cell 135:549-60. 2008
    ..Our results demonstrate that neurofibromin modulates ERK/synapsin I-dependent GABA release, which in turn modulates hippocampal LTP and learning...
  80. doi Increased c-Jun-NH2-kinase signaling in neurofibromatosis-1 heterozygous microglia drives microglia activation and promotes optic glioma proliferation
    Girish C Daginakatte
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Cancer Res 68:10358-66. 2008
    Neurofibromatosis-1 (NF1) is a common tumor predisposition syndrome in which affected individuals develop benign and malignant tumors...
  81. pmc Malignant astrocytomas originate from neural stem/progenitor cells in a somatic tumor suppressor mouse model
    Sheila Alcantara Llaguno
    Department of Developmental Biology, The University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390, USA
    Cancer Cell 15:45-56. 2009
    ..reported mouse models based on conditional inactivation of the human astrocytoma-relevant tumor suppressors p53, Nf1, and Pten, wherein through somatic loss of heterozygosity, mutant mice develop tumors with 100% penetrance...
  82. doi The neurofibromatosis type 1 tumor suppressor controls cell growth by regulating signal transducer and activator of transcription-3 activity in vitro and in vivo
    Sutapa Banerjee
    Department of Neurology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Cancer Res 70:1356-66. 2010
    ..The NF1 gene product neurofibromin negatively regulates Ras and mammalian target of rapamycin (mTOR) signaling, prompting clinical trials to ..
  83. pmc Ultrastructural characterization of the optic pathway in a mouse model of neurofibromatosis-1 optic glioma
    K Y Kim
    Center for Research in Biological Systems, National Center for Microscopy and Imaging Research and Department of Neurosciences, University of California San Diego School of Medicine, La Jolla, CA 92037, USA
    Neuroscience 170:178-88. 2010
    ..to investigate the progression of changes in retinal ganglion cells and optic nerve glia in neurofibromatosis-1 (NF1) genetically-engineered mice with optic glioma...
  84. pmc Reduced striatal dopamine underlies the attention system dysfunction in neurofibromatosis-1 mutant mice
    Jacquelyn A Brown
    Department of Neurology, Washington University School of Medicine, St Louis, MO, USA
    Hum Mol Genet 19:4515-28. 2010
    ..and behavioral abnormalities are among the most common clinical problems in children with the neurofibromatosis-1 (NF1) inherited cancer syndrome...
  85. pmc Primary osteopathy of vertebrae in a neurofibromatosis type 1 murine model
    Wei Zhang
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA
    Bone 48:1378-87. 2011
    Neurofibromatosis type 1 (NF1) is a common autosomal dominant genetic disorder caused by mutation of the NF1 tumor suppressor gene. Spinal deformities are common skeletal manifestations in patients with NF1...
  86. pmc Neurofibromin (Nf1) is required for skeletal muscle development
    Nadine Kossler
    FG Development and Disease, Max Planck Institute for Molecular Genetics, Ihnestrasse 73, Berlin, Germany
    Hum Mol Genet 20:2697-709. 2011
    ..type 1 (NF1) is a multi-system disease caused by mutations in the NF1 gene encoding a Ras-GAP protein, neurofibromin, which negatively regulates Ras signaling...
  87. pmc Perinatal or adult Nf1 inactivation using tamoxifen-inducible PlpCre each cause neurofibroma formation
    Debra A Mayes
    Cincinnati Children s Hospital Medical Center Hoxworth Blood Center, University of Cincinnati, Cincinnati, Ohio, USA
    Cancer Res 71:4675-85. 2011
    Plexiform neurofibromas are peripheral nerve sheath tumors initiated by biallelic mutation of the NF1 tumor suppressor gene in the Schwann cell lineage...
  88. pmc Mosaic analysis with double markers reveals tumor cell of origin in glioma
    Chong Liu
    Institute of Molecular Biology, University of Oregon, Eugene, OR 97403, USA
    Cell 146:209-21. 2011
    ..Here, we use mosaic analysis with double markers (MADM) in mice to model gliomagenesis by initiating concurrent p53/Nf1 mutations sporadically in neural stem cells (NSCs)...
  89. pmc Mice lacking Nf1 in osteochondroprogenitor cells display skeletal dysplasia similar to patients with neurofibromatosis type I
    Weixi Wang
    Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    Hum Mol Genet 20:3910-24. 2011
    Mutations in NF1 cause neurofibromatosis type I (NF1), a disorder characterized, among other clinical manifestations, by generalized and focal bony lesions...
  90. pmc PET imaging for attention deficit preclinical drug testing in neurofibromatosis-1 mice
    Jacquelyn A Brown
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Exp Neurol 232:333-8. 2011
    ..abnormalities represent a significant barrier to scholastic achievement in children with neurofibromatosis-1 (NF1)...
  91. pmc The haploinsufficient hematopoietic microenvironment is critical to the pathological fracture repair in murine models of neurofibromatosis type 1
    Xiaohua Wu
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, United States of America
    PLoS ONE 6:e24917. 2011
    Germline mutations in the NF1 tumor suppressor gene cause neurofibromatosis type 1 (NF1), a complex genetic disorder with a high predisposition of numerous skeletal dysplasias including short stature, osteoporosis, kyphoscoliosis, and ..
  92. doi Dorsal root ganglia isolated from Nf1+/- mice exhibit increased levels of mRNA expression of voltage-dependent sodium channels
    K E Hodgdon
    Department of Pharmacology and Toxicology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    Neuroscience 206:237-44. 2012
    We reported previously that sensory neurons isolated from mice with a heterozygous mutation of the Nf1 gene (Nf1+/-) exhibited greater excitability and increased sodium current densities compared with wildtype mice...
  93. pmc ERK inhibition rescues defects in fate specification of Nf1-deficient neural progenitors and brain abnormalities
    Yuan Wang
    Division of Molecular Medicine and Genetics, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI 48109, USA
    Cell 150:816-30. 2012
    ..1 (NF1), an NCFC syndrome, is caused by loss-of-function heterozygous mutations in the NF1 gene, which encodes neurofibromin, a RAS GTPase-activating protein...
  94. pmc Assessment of pain and itch behavior in a mouse model of neurofibromatosis type 1
    Daniel E O'Brien
    Neuroscience Program, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Pain 14:628-37. 2013
    Neurofibromatosis type 1 (NF1) is characterized primarily by tumor formation in the nervous system, but patients report other neurological complications including pain and itch...
  95. doi Hyperactive transforming growth factor-β1 signaling potentiates skeletal defects in a neurofibromatosis type 1 mouse model
    Steven D Rhodes
    Department of Anatomy and Cell Biology, Indiana University School of Medicine, Indianapolis, IN, USA Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indiana University School of Medicine, Indianapolis, IN, USA
    J Bone Miner Res 28:2476-89. 2013
    ..Reexpression of the human, full-length neurofibromin guanosine triphosphatase (GTPase)-activating protein (GAP)-related domain (NF1 GRD) in primary Nf1-deficient ..
  96. pmc Multiscale, converging defects of macro-porosity, microstructure and matrix mineralization impact long bone fragility in NF1
    Jirko Kühnisch
    Institute for Medical Genetics and Human Genetics, Charite Universitatsmedizin Berlin, Berlin, Germany FG Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany
    PLoS ONE 9:e86115. 2014
    ..debilitating focal skeletal dysplasias is a frequent observation in the Mendelian disease Neurofibromatosis type 1 (NF1)...
  97. pmc N-type calcium current, Cav2.2, is enhanced in small-diameter sensory neurons isolated from Nf1+/- mice
    J H Duan
    Department of Pharmacology and Toxicology, School of Medicine, Indiana University, Indianapolis, IN 46202, USA
    Neuroscience 270:192-202. 2014
    ..We reported previously that sensory neurons isolated from a mouse model with a heterozygous mutation of the Nf1 gene (Nf1+/-) exhibited both greater excitability and evoked release of neuropeptides compared to wildtype mice...
  98. ncbi Mlh1 deficiency accelerates myeloid leukemogenesis in neurofibromatosis 1 (Nf1) heterozygous mice
    D H Gutmann
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    Oncogene 22:4581-5. 2003
    ..Recent studies have suggested that one of the targets of MMR is the neurofibromatosis 1 (NF1) gene...
  99. ncbi Cloning of a cDNA encoding the smallest neurofilament protein from the rat
    J P Julien
    Biochim Biophys Acta 825:398-404. 1985
    ..Blot analysis indicates that the cDNA is derived from a single neurofilament gene that codes for two different poly(A)+ mRNA species...
  100. ncbi Vascular system defects and neuronal apoptosis in mice lacking ras GTPase-activating protein
    M Henkemeyer
    Programme in Molecular Biology and Cancer, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada
    Nature 377:695-701. 1995
    ..Thus rasGAP and neurofibromin act together to regulate Ras activity during embryonic development.
  101. ncbi Human chromosome 17 comparative anchor loci are conserved on bovine chromosome 19
    Y P Yang
    Department of Veterinary Pathobiology, Texas A and M University, College Station 77843, USA
    Genomics 27:293-7. 1995
    Eight comparative anchor loci on human chromosome 17, TP53, CHRNB1, THRA1, CRYB1, NF1, MPO, MYL4, and P4HB, were mapped to bovine chromosome 19 using bovine x hamster and bovine x mouse hybrid somatic cell lines...

Research Grants14

  1. NEUROFIBROMIN AS A NEGATIVE REGULATOR FOR ASTROCYTES
    David Gutmann; Fiscal Year: 2002
    DESCRIPTION (Adapted from applicant's abstract): Neurofibromatosis 1 (NF1) is a comm mon autosomal dominant disorder in which affected individuals develop benign and malignant tumors...
  2. FUNCTIONAL ANALYSIS OF NF2 GENE MUTATIONS
    David Gutmann; Fiscal Year: 2003
    ..Our ability to design rational therapies for schwannomas and meningiomas is dependent on an improved understanding of the mechanisms by which loss of merlin expression and function promotes tumor formation. ..
  3. PROTEIN 4.1 TUMOR SUPPRESSORS IN MENINGIOMA PATHOGENESIS
    David Gutmann; Fiscal Year: 2003
    ..and (3) analyzing the ability of DAL-1 to impair cell growth and motility. These studies are collectively designed to define the role of this novel family of growth regulators in meningioma tumorigenesis and progression. ..
  4. Neurofibromatosis Foundation International Consortium
    David Gutmann; Fiscal Year: 2003
    ..In addition to updating investigators working on NF 1 and NF2 on the latest research developments, this meeting will help to identify critical gaps in our knowledge as ..
  5. PRECLINICAL MODELS FOR HUMAN ASTROCYTOMAS
    David Gutmann; Fiscal Year: 2004
    ..abstract_text> ..
  6. Identification and Preclinical Evaluation of New Brain Tumor Therapies
    David Gutmann; Fiscal Year: 2007
    ..of neoplastic NF1-deficient astrocytes, is the second most common tumor in individuals affected with the neurofibromatosis 1 (NF1) tumor predisposition syndrome...
  7. Molecular Determinants of Neural Stem Cell Function
    David Gutmann; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  8. GENETICS OF A TRANSGENIC MOUSE MODEL FOR NEUROBLASTOMA
    William Weiss; Fiscal Year: 2004
    ..Given the poor outcome associated with childhood neuroblastoma, this work has the potential to increase our understanding of the pathogenesis of neuroblastoma, and may result in improved therapies for children with this disorder. ..
  9. Imaging Kinase Activity In Vivo
    William Weiss; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  10. Ras-PI3K Pathway in Nf1-/-Hematopoiesis and Leukemia
    David Ingram; Fiscal Year: 2007
    ..Children with NF1 are predisposed to developing juvenile myelomonocytic leukemia (JMML). Neurofibromin, the protein product of NF1, is a negative regulator of p21ras activity...
  11. Targeting Vasculogenesis and Interstitial Hypertension in Neurofibromatosis Type1
    Ugur Ozerdem; Fiscal Year: 2007
    ..tumors (MPNST) are the most common malignant tumor, and the leading cause of mortality in neurofibromatosis type 1 (NF1). Two critical issues in MPNST are neovascularization and high interstitial fluid pressure (IFP)...
  12. 2007 Neurofibromatosis Conference
    Karen Cichowski; Fiscal Year: 2007
    ..This will promote to the research community at large the status of NF research, the link with other cancers and neurological disorders and the key barriers to be addressed. [unreadable] [unreadable] [unreadable]..
  13. Actions of GTPase-Activating Proteins on Sensory Neurons
    Cynthia M Hingtgen; Fiscal Year: 2010
    ..The activity of this cascade is regulated by GTPase activating protiens (GAPs), such as neurofibromin and SynGAP...
  14. Cellular and molecular basis of malignant astrocytoma
    Yuan Zhu; Fiscal Year: 2010
    ..Specifically, we will determine (1) whether tumor suppressors p53 and Neurofibromatosis type 1 (NF1) play physiological roles in regulating NSC proliferation, apoptosis and differentiation in the adult brain and (2) ..