Transgenic mice as research tools in neurocarcinogenesis

Reference

• Aguzzi A., Brandner S., Isenmann S., Steinbach J. P., Sure U. Transgenic and gene disruption techniques in the study of neurocarcinogenesis. Glia 1995; 15: 348–364
   PubMed Web of Science ®Google Scholar
• Aguzzi A., Ellmeier W., Weith A. Dominant and recessive molecular changes in neuroblastomas. Brain-Pathol 1992; 2: 195–208
   Google Scholar
• Aguzzi A., Kleihues P., Heckl K., Wiestler OD. Cell type-specific tumor induction in neural transplants by retrovirus-mediated oncogene transfer. Oncogene 1991; 6: 113–118
   PubMed Web of Science ®Google Scholar
• Aguzzi A., Wagner E. F., Williams R. L., Courtneidge SA. Sympathetic hyperplasia and neuroblastomas in transgenic mice expressing polyoma middle T antigen. New-Biol 1990; 2: 533–543
   PubMedGoogle Scholar
• Aguzzi A., Weber T., Paulus W. Choroid plexus tumors. Pathology and Genetics of Brain Tumors, P Kleihues, W Cavenee. Int. Soc. of Neuropathology, (Lyon 1997
   Google Scholar
• Alarid E. T., Windle J. J., Whyte D. B., Mellon PL. Immortalization of pituitary cells at discrete stages of development by directed oncogenesis in transgenic mice. Development 1996; 122: 3319–3329
   PubMed Web of Science ®Google Scholar
• Barlow C., Hirotsune S., Paylor R., Liyanage M., Eckhaus M., Collins F., Shiloh Y., Crawley J. N., Ried T., Tagle D., Wynshaw Boris A. Atm-deficient mice: a paradigm of ataxia telangiectasia. Cell 1996; 86: 159–171
   PubMed Web of Science ®Google Scholar
• Baron U., Gossen M., Bujard H. Tetracycline controlled transcription in eukaryotes: novel transac-tivators with graded transactivation potential. Nucleic Acids Research 1997; 25: 2723–2729
   PubMedGoogle Scholar
• Belloni E., Muenke M., Roessler E., Traverso G., Siegel Bartelt J., Frumkin A., Mitchell H. F., Dords Keller H., Helms C., Hing A. V., Heng H. H., Koop B., Martindale D., Rommens J. M., Tsui L. C., Scherer SW. Identification of Sonic hedgehog as a candidate gene responsible for holoprosencephaly. Nat-Genet 1996; 14: 353–356
   PubMed Web of Science ®Google Scholar
• Bohl D., Heard J.M. Modulation of erythropoietin delivery from engineered muscles in mice. Human Gene Therapy 1997; 8: 195–204
   PubMedGoogle Scholar
• Bollag G., Clapp D. W., Shih S., Adler F., Zhang Y. Y., Thompson P., Lange B. J., Freedman M. H., Mccormick F., Jacks T., Shannon K. Loss of NFl results in activation of the Ras signaling pathway and leads to aberrant growth in haematopoietic cells. Nat-Genet 1996; 12: 144–148
   PubMed Web of Science ®Google Scholar
• Boulter C. A., Aguzzi A., Williams R. L., Wagner E. F., Evans M. J., Beddington R. Expression of v-src induces aberrant development and twinning in chimaeric mice. Development 1991; 111: 357–366
   PubMedGoogle Scholar
• Bowman T., Symonds H., Gu L., Yin C., Oren M., Van Dyke T. Tissue-specific inactivation of p53 tumor suppression in the mouse. Genes-Dev 1996; 10: 826–835
   Google Scholar
• Brannan C. I., Perkins A. S., Vogel K. S., Ratner N., Nordlund M. L., Reid S. W., Buchberg A. M., Jenkins N. A., Parada L. F., Copeland NG. Targeted disruption of the neurofibromatosis type-1 gene leads to developmental abnormalities in heart and various neural crest-derived tissues. Genes-Dev 1994; 8: 1019–1029
   PubMed Web of Science ®Google Scholar
• Brodeur G.M. Molecular basis for heterogeneity in human neuroblastomas. Eur J Cancer 1995; 4: 505–510
   Google Scholar
• Brodeur G. M., Fong C. T., Morita M., Griffith R., Hayes F. A., Seeger RC. Molecular analysis and clinical significance of N-myc amplification and chromosome 1p monosomy in human neuroblastomas. Prog Clin Biol Res 1988a; 271: 3–15
   PubMedGoogle Scholar
• Brodeur G. M., Maris J. M., Yamashiro D. J., Hogarty M. D., White PS. Biology and genetics of human neuroblastomas. Journal of Pediatric Hematology Oncology 1997; 19: 93–101
   PubMed Web of Science ®Google Scholar
• Brodeur G. M., Seeger R. C., Barrett A., Berthold F., Castleberry R. P., D'Angio G., Bernardi B., Evans A. E., Favrot M., Freeman A.I., et al. International criteria for diagnosis, staging, and response to treatment in patients with neuroblastoma (see comments). J-Clin-Oncol 1988b; 6: 1874–1881
   PubMed Web of Science ®Google Scholar
• Brodeur G. M., Seeger R. C., Schwab M., Varmus H. E., Bishop JM. Amplifiction of N-myc in untreated human neuroblastomas correlates with advanced disease stage. Science 1984; 224: 1121–1124
   PubMed Web of Science ®Google Scholar
• Brodeur G. M., Seeger R. C., Schwab M., Varmus H. E., Bishop JM. Amplification of N-myc sequences in primary human neuroblastomas: correlation with advanced disease stage. Prog Clin Biol Res 1985; 175: 105–113
   PubMedGoogle Scholar
• Bronson S. K., Plaehn E. G., Kluckman B.C.D., Hagaman J. R., Maeda N., Smithies O. Single-copy transgenic mice with chosen-site integration [see comments]. Proc Natl Acad Sci USA 1996; 93: 9067–9072
   PubMedGoogle Scholar
• Brugarolas J., Jacks T. Double indemnity: p53, brca and cancer. Nature Medicine 1997; 3: 721–722
   PubMedGoogle Scholar
• Carlson K. M., Dou S., Chi D., Scavarda N., Toshima K., Jackson C. E., Wells S.A., Jr, Goodfellow P. J., Donis Keller H. Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci USA 1994; 91: 1579–1583
   PubMed Web of Science ®Google Scholar
• Carney M. E., O' Reilly R. C., Sholevar B., Buikova O. I., Lowry L. D., Keane W. M., Margolis F. L., Rothstein JL. Expression of the human Achaete-scute 1 gene in olfactory neuroblastoma (esthesioneuroblastoma). J Neurooncol 1995; 26: 35–43
   Google Scholar
• Chen D. F., Schneider G. E., Martinou J. C., Tonegawa S. Bel 2 promotes regeneration of severed axons in mammalian ens. Nature 1997; 385: 434–439
   PubMed Web of Science ®Google Scholar
• Chi D. D., Toshima K., Donis Keller H., Wells S.A., Jr. Predictive testing for multiple endocrine neoplasia type 2A (MEN 2A) based on the detection of mutations in the RET protooncogene. Surgery 1994; 116: 132–133, discussion 124–132
   Google Scholar
• Chiang C., Litingtung Y., Lee E., Young K. E., Corden J. L., Wesphal H., Beachy PA. Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene function. Nature 1996; 383: 407–413
   PubMed Web of Science ®Google Scholar
• Christofoi G., Naik P., Hanahan D. Deregulation of both imprinted and expressed alleles of the insulin-like growth factor 2 gene during beta-cell tumorigenesis. Nat Genet 1995; 10: 196–201
   Google Scholar
• Chu T.M. Molecular diagnosis of pancreas carcinoma. Journal of Clinical Laboratory Analysis 1997; 11: 225–231
   PubMed Web of Science ®Google Scholar
• Colucci, D'Amato G. L., Santelli G., Chiappetta G.A.D.A., Mineo A., Manzo G., Vecchio G., De Franciscis V. Dbl expression driven by the neuron specific enolase promoter induces tumor formation in transgenic mice with a p53(+/-) genetic background. Biochem Biophys Res Commun 1995; 216: 762–770
   Google Scholar
• Corral J., Lavenir I., Impey H., Warren A. J., Forster A., Larson T. A., Bell S., Mckenzie A. N., King G., Rabbitts TH. An M11-AF9 fusion gene made by homologous recombination causes acute leukemia in chimeric mice: a method to create fusion oncogenes. Cell 1996; 85: 853–861
   PubMed Web of Science ®Google Scholar
• Danks R. A., Orian J. M., Gonzales M. F., Tan S. S., Alexander B., Mikoshiba K., Kaye AH. Transformation of astrocytes in transgenic mice expressing SV40 T antigen under the transcriptional control of the glial fibrillary acidic protein promoter. Cancer Res 1995; 55: 4302–4310
   PubMed Web of Science ®Google Scholar
• Delellis R. A., Dayal Y., Tischler A. S., Lee A. K., Wolfe HJ. Multiple endocrine neoplasia (MEN) syndromes: cellular origins and interrelationships. Int Rev Exp Pathol 1986; 28: 163–215
   PubMedGoogle Scholar
• Dickinson M. E., Krumlauf R., Mcmahon AP. Evidence for a mitogenic effect of Wnt-1 in the developing mammalian central nervous system. Development 1994; 120: 1453–1471
   PubMed Web of Science ®Google Scholar
• Disanto J. P., Muller W., Guy Grand D., Fisher A., Rajewsky K. Lymphoid development in mice with a targeted deletion of the interleukin 2 receptor gamma chain. Proc Natl Acad Sci USA 1995; 92: 377–381
   PubMed Web of Science ®Google Scholar
• Donehower L.A. Genetic instability in animal tumorigenesis models. Cancer Surveys 1997; 29: 329–352
   PubMedGoogle Scholar
• Donehower L.A. The p53 deficient mouse: a model for basic and applied cancer studies. Seminars in Cancer Biology 1996; 7: 269–278
   PubMedGoogle Scholar
• Donehower L. A., Godley L. A., Aldaz C. M., Pyle R., Shi Y. P., Pinkel D., Gray J., Bradley A., Medina D., Varmus HE. Deficiency of p53 accelerates mammary tumorigenesis in Wnt-1 transgenic mice and promotes chromosomal instability. Genes Dev 1995; 9: 882–895
   PubMed Web of Science ®Google Scholar
• Donis Keller H. The RET proto-oncogene and cancer. J Intern Med 1995; 238: 319–325
   Google Scholar
• Donis, Keller H., Dou S., Chi D., Carlson K. M., Toshima K., Lairmore T. C., Howe J. R., Moley J. F., Goodfellow P., Wells S.A., Jr. Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet 1993; 2: 851–856
   Google Scholar
• Durbec P., Marcos Gutierrez C. V., Kilkenny C., Grigoriou M., Wartiowaara K., Suvanto P., Smith D., Ponder B., Costantini F., Saarma M., et al. GDNF signalling through the Ret receptor tyrosine kinase (see comments). Nature 1996; 381: 789–793
   PubMed Web of Science ®Google Scholar
• Eng C., Smith D. P., Mulligan L. M., Healey C. S., Zvelebil M. J., Stonehouse T. J., Ponder M. A., Jackson C. E., Waterfield M. D., Ponder B.A. A novel point mutation in the tyrosine kinase domain of the RET proto-oncogene in sporadic medullary thyroid carcinoma and in a family with FMTC. Oncogene 1995; 10: 509–513
   PubMed Web of Science ®Google Scholar
• Eng C., Smith D. P., Mulligan L. M., Nagai M. A., Healey C. S., Ponder M. A., Gardner E., Scheumann G. F., Jackson C. E., Tunnacliffe A., et al. Point mutation within the tyrosine kinase domain of the RET proto-oncogene in multiple endocrine neoplasia type 2B and related sporadic tumours (published erratum appears in. Hum Mol Genet Apr 1994; 3((4))237–241
   Google Scholar
• Enjoji M. Human HE2 (microB) and microA motifs show the same function as whole IgH intronic enhancer in transgenic mice. Mol Cell Biochem 1994; 137: 33–37
   Google Scholar
• Enjoji M., Iwaki T., Nawata H., Watanabe T. IgH intronic enhancer element HE2 (mu B) functions as a cis-activator in choroid plexus cells at the cellular level as well as in transgenic mice. J Neurochem 1995; 64: 961–966
   Google Scholar
• Fan H. R., Oro A. E., Scott M. P., Khavari PA. Induction of basal cell carcinoma features in transgenic human skin expressing sonic hedgehog. Nature Medicine 1997; 3: 788–792
   PubMed Web of Science ®Google Scholar
• Feigenbaum L., Fujita K., Collins F. S., Jay G. Repression of the NF1 gene by Tax may explain the development of neuronbromas in human T-lympho-tropic virus type 1 transgenic mice. J Virol 1996; 70: 3280–3285
   PubMed Web of Science ®Google Scholar
• Ferreira A., Kosik KS. Accelerated neuronal differentiation induced by p53 suppression. J Cell Sci 1996; 109: 1509–1516
   Google Scholar
• Fong C. T., Dracopoli N. C., White P. S., Merrill P. T., Griffith R. C., Housman D. E., Brodeur GM. Loss of heterozygosity for the short arm of chromosome 1 in human neuroblastomas: correlation with N-myc amplification. Proc Natl Acad Sci USA 1989; 86: 3753–3757
   PubMed Web of Science ®Google Scholar
• Fong C. T., White P. S., Peterson K., Sapienza C., Cavenee W. K., Kern S. E., Vogelstein B., Cantor A. B., Look A. T., Brodeur GM. Loss of heterozygosity for chromosomes 1 or 14 defines subsets of advanced neuroblastomas. Cancer Res 1992; 52: 1780–1785
   Google Scholar
• Frank C. J., Mcclatchey K. D., Devaney K. O., Carey TE. Evidence that loss of chromosome 18q is associated with tumor progression. Cancer Research 1997; 57: 824–827
   PubMed Web of Science ®Google Scholar
• Franks R. R., Rencic A., Gordon J., Zoltick P. W., Curtis M., Knobler R. L., Khalili K. Formation of undiffer-entiated mesenteric tumors in transgenic mice expressing human neurotropic polymavirus early protein. Oncogene 1996; 12: 2573–2578
   Google Scholar
• Fritschy J. M., Brandner S., Aguzzi A., Koedood M., Luscher B., Mitchell PJ. Brain cell type specificity and gliosis-induced activation of the human cytomegalo-virus immediate-early promoter in transgenic mice. J Neurosci 1996; 16: 2275–2282
   Google Scholar
• Fung K. M., Chikaraishi D. M., Suri C., Theuring F., Messing A., Albert D. M., Lee V. M., Trojanowski J.Q. Molecular phenotype of simian virus 40 large T antigen-induced primitive neuroectodermal tumors in four different lines of transgenic mice. Lab Invest 1994; 70: 114–124
   Google Scholar
• Fung K. M., Lee V. M., Trojanowski JQ. Dynamics of cell proliferation and cell death during the emergence of primitive neuroectodermal tumors of the immature central nervous system in transgenic mice. Am J Pathol 1995; 146: 1376–1387
   Google Scholar
• Fung K. M., Trojanowski J.Q. Animal models of medulloblastomas and related primitive neuroectoder-mal tumors. A review. J Neuropathol Exp Neurol 1995; 54: 285–296
   PubMed Web of Science ®Google Scholar
• Goodrich L. V., Milenkovitch L., Higgins K. M., Scott M.P. Altered Neural Fates and Medulloblastoma in Mouse patched Mutants. Science 1997; 277: 1109–1113
   PubMed Web of Science ®Google Scholar
• Gowen L. C., Johnson B. L., Latour A. M., Sulik K. K., Koler BH. Brcal deficiency results in early embryonic lethality characterized by neuroepithelial abnormalities. Nat Genet 1996; 12: 191–194
   PubMed Web of Science ®Google Scholar
• Gu H., Zou Y. R., Rajewsky K. ndependent control of immunoglobulin switch recombination at individual switch regions evidenced through Cre-loxP-mediated gene targeting. Cell 1993; 73: 1155–1164
   PubMed Web of Science ®Google Scholar
• Gunther T., Struwe M., Aguzzi A., Schughart K. Open brain, a new mouse mutant with several neural tube defects, shows altered gene expression patterns in the developing spinal cord. Development 1994; 120: 3119–3130
   Google Scholar
• Guo H. F., The I., Hannan F., Bernards A., Zhong Y. Requirement of Drosophila NF1 for activation of adenylyl cyclase by PACAP38-like neuropeptides. Science 1997; 276: 795–798
   Google Scholar
• Hakem R., De La Pompa J. L., Sirard C., Mo R., Woo M., Hakem A., Wakeham A., Potter J., Reitmair A., Billia F., Firpo E., Hui C. C., Roberts J., Rossant J., Mak TW. The tumor suppressor gene Brcal is required for embryonic cellular proliferation in the mouse. Cell 1996; 85: 1009–1023
   PubMed Web of Science ®Google Scholar
• Hakem R., Delapompa J. L., Eli A., Potter J., Mak TW. Partial rescue of brca1(56) early embryonic lethality by p53 or p21 null mutation. Nature Genetics 1997; 16: 298–302
   PubMed Web of Science ®Google Scholar
• Hanahan D. Transgenic mice as probes into complex systems. Science (AAAS) 1989; 246: 1265–1275
   PubMed Web of Science ®Google Scholar
• Harvey M., Vogel H., Morris D., Bradley A., Bernstein A., Donehower LA. A mutant p53 transgene accelerates tumour development in heterozygous but not nullizygous p53-deficient mice. Nat Genet 1995; 9: 305–311
   PubMed Web of Science ®Google Scholar
• Helseth A., Haug E., Nesland J. M., Siegal G. P., Fodstad O., Bautch VL. Endocrine and metabolic characteristics of polyoma large T transgenic mice that develop ACTH-producing pituitary tumors. J Neurosurg 1995; 82: 879–885
   Google Scholar
• Helseth A., Siegal G. P., Haug E., Bautch VL. Transgenic mice that develop pituitary tumors. A model for Cushing's disease. Am J Pathol 1992; 140: 1071–1080
   Google Scholar
• Henkemeyer M., Rossi D. J., Holmyard D. P., Puri M. C., Mbamalu G., Harpal K., Shih T. S., Jacks T., Pawson T. Vascular system defects and neuronal apoptosis in mice lacking ras GTPase-activating protein. Nature 1995; 377: 695–701
   PubMed Web of Science ®Google Scholar
• Hilberg F., Aguzzi A., Howells N., Wagner EF. c-jun is essential for normal mouse development and hepatogenesis [published erratum appears in. Nature 1993; 366: 179–181, 368
   Google Scholar
• Hinrichs S. H., Nerenberg M., Reynolds R. K., Khoury G., Jay G. A transgenic mouse model for human neurofibromatosis. Science 1987; 237: 1340–1343
   PubMed Web of Science ®Google Scholar
• Holash J. A., Harik S. I., Perry G., Stewart PA. Barrier properties of testis microvessels. Proc Natl Acad Sci USA 1993; 90: 11069–11073
   Google Scholar
• Holm R., Helseth A., Nesland J. M., Bautch VL. ACTH-producing pituitary tumors in transgenic mice. An ultrastructural and immunoelectron microscopic study. J Submicrosc Cytol Pathol 1993; 25: 29–36
   Google Scholar
• Huh S. O., Park D. H., Cho J. Y., Joh T. H., Son JH. A 6.1 kb 5′ upstream region of the mouse tryptophan hydroxylase gene directs expression of E. coli lacZ to major serotonergic brain regions and pineal gland in transgenic mice. Brain Res Mol Brain Res 1994; 24: 145–152
   Google Scholar
• Jacks T., Remington L., Williams B. O., Schmitt E. M., Halachmi S., Bronson R. T., Weinberg RA. Tumor spectrum analysis in p53-mutant mice. Curr Biol 1994a; 4: 1–7
   PubMed Web of Science ®Google Scholar
• Jacks T., Shih T. S., Schmitt E. M., Bronson R. T., Bernards A., Weinberg RA. Tumour predisposition in mice heterozygous for a targeted mutation in Nfl. Nat Genet 1994b; 7: 353–361
   PubMed Web of Science ®Google Scholar
• Jiang Z., Zacksenhaus E., Gallie B. L., Phillips RA. The retinoblastoma gene family is differentially expressed during embryogenesis. Oncogene 1997; 14: 1789–1797
   PubMed Web of Science ®Google Scholar
• Jochum W., Weber T., Frye S., Hunsmann G., Luke G., Aguzzi A. Detection of JC virus by anti-VPl immunohistochemistry in brains with progressive multifocal leukoencephalopathy. Acta Neuropathol 1997; 94: 226–231
   Google Scholar
• Johnson R. L., Rothman A. L., Xie J., Goodrich L. V., Bare J. W., Bonifas J. M., Quinn A. G., Myers R. M., Cox D. R., Epstein E.H., Jr, Scott MP. Human homolog of patched, a candidate gene for the basal cell nevus syndrome. Science 1996; 272: 1668–1671
   PubMed Web of Science ®Google Scholar
• Kim H. A., Rosenbaum T., Marchionni M. A., Ratner N., Declue JE. Schwann cells from neurofibromin deficient mice exhibit activation of p21ras, inhibition of cell proliferation and morphological changes. Oncogene 1995; 11: 325–335
   PubMedGoogle Scholar
• Kinzler T.C.W, Vogelstein B. Cancer-susceptibility genes. Gatekeepers and caretakers (news; comment). Nature 1997; 386: 761, 763
   PubMed Web of Science ®Google Scholar
• Kistner A., Gossen M., Zimmermann F., Jerecis J., Ullmer C., Lubbert H., Bujard H. Doxycycline-mediated quantitative and tissue-specific control of gene expression in transgenic ‘mice. Proc Natl Acad Sci USA 1996; 93: 10933–10938
   PubMed Web of Science ®Google Scholar
• Krajewski S., Krajewska M., Ehrmann J., Sikorska M., Lach B., Chatten J., Reed JC. Immunohistochemical analysis of bcl 2, bcl x., mcl 1, and bax in tumors of central and peripheral nervous system origin. American Journal of Pathology 1997; 150: 805–814
   PubMed Web of Science ®Google Scholar
• Krajewski S., Krajewska M., Shabaik A., Wang H. G., Me S., Fong L., Reed JC. Immunohistochemical analysis of in vivo patterns of Bcl-X expression. Cancer Res 1994; 54: 5501–5507
   PubMed Web of Science ®Google Scholar
• Krajewski S., Mai J. K., Krajewska M., Sikorska M., Mossakowski M. J., Reed JC. Upregulation of bax protein levels in neurons following cerebral ischemia. J Neurosci 1995; 15: 6364–6376
   PubMed Web of Science ®Google Scholar
• Lagna G., Hata A., Hemmati Brivanlou A., Massague J. Partnership between DPC4 and SMAD proteins in TGF-beta signalling pathways. Nature 1996; 383: 832–836
   PubMed Web of Science ®Google Scholar
• Largaespada D. A., Brannan C. I., Jenkins N. A., Copeland NG. Nfl deficiency causes Ras-mediated granulo-cyte/macrophage colony stimulating factor hypersen-sitivity and chronic myeloid leukaemia. Nat Genet 1996; 12: 137–143
   PubMed Web of Science ®Google Scholar
• Leao M., Da Silva ML. Evidence of central nervous system involvement in Watson syndrome. Pediatr Neurol 1995; 12: 252–254
   Google Scholar
• Lee F. S., Lane T. F., Kuo A., Shackleford G. M., Leder P. Insertional mutagenesis identifies a member of the Wnt gene family as a candidate oncogene in the mammary epithelium of int-2/Fgf-3 transgenic mice. Proc Natl Acad Sci USA 1995; 92: 2268–2272
   PubMed Web of Science ®Google Scholar
• Li C., Schwabe JWR, Banayo E., Evans RM. Coexpression of nuclear receptor partners increases their solubility and biological activities. Proceedings of The National Academy of Sciences of The United States of America 1997a; 94: 2278–2283
   PubMedGoogle Scholar
• Li Q., Clegg C., Peterson K., Shaw S., Raich N., Stamatoyannopoulos G. Binary transgenic mouse model for studying the trans control of globin gene switching: evidence that GATA-1 is an in vivo repressor of human epsilon gene expression. Proc Natl Acad Sci USA 1997b; 94: 2444–2448
   PubMedGoogle Scholar
• Li Z. W., Stark G., Gotz J., Rulicke T., Muller U., Weissmann C. Generation of mice with a 200 kb amyloid precursor protein gene deletion by Cre recombinase-mediated site-specific recombination in embryonic stem cells. Proc Natl Acad Sci USA 1996; 93: 6158–6162
   Google Scholar
• Liu C. Y., Flesken Nikitin A., Li S., Zeng Y., Lee WH. Inactivation of the mouse Brcal gene leads to failure in the morphogenesis of the egg cylinder in early postimplantation development. Genes Dev 1996; 10: 1835–1843
   PubMed Web of Science ®Google Scholar
• Ludwig T., Chapman D. L., Papaioannou V. E., Efstratiadis A. Targeted mutations of breast cancer susceptibility gene homologs in mice: lethal phenotypes of brcal, brca2, brcal/brca2, brcal/p53, and brca2/p53 nullizygous embryos. Genes and Development. 1997; 11: 1226–1241
   PubMed Web of Science ®Google Scholar
• Macleod K. F., Hu Y., Jacks T. Loss of Rb activates both p53-dependent and independent cell death pathways in the developing mouse nervous system. EMBO J 1996; 15: 6178–6188
   PubMed Web of Science ®Google Scholar
• Magrio V., Davey R. A., Zuo Y., Cunningham J. M., Tabin CJ. Biochemical evidence that patched is the Hedgehog receptor (see comments). Nature 1996; 384: 176–179
   Google Scholar
• Maris J. M., Kyemba S. M., Rebbeck T. R., White P. S., Sulman E. P., Jensen S. J., Allen C., Biegel J. A., Yanofsky R. A., Feldman G. L., Brodeur GM. Familial predisposition to neuroblastoma does not map to chromosome band Ip36. Cancer Res 1996; 56: 3421–3425
   Google Scholar
• Mccarthy S. A., Symonds H. S., Vandyke T. Regulation of apoptosis in transgenic mice by simian virus 40 T antigen-mediated inactivation of p53. Proc Natl Acad Sci USA 1994; 91: 3979–3983
   PubMed Web of Science ®Google Scholar
• Mcmahon A. P., Chuang PT. Hedgehogs in the clinic. Nat Med 1996; 2: 1308–1310
   Google Scholar
• Merry D. E., Korsmeyer SJ. Bcl-2 gene family in the nervous system. Annu Rev Neurosci 1997; 20: 245–267
   PubMed Web of Science ®Google Scholar
• Messing A., Chen H. Y., Palmiter R. D., Brinster RL. Peripheral neuropathies, hepatocellular carcinomas and islet cell adenomas in transgenic mice. Nature 1985; 316: 461–463
   PubMed Web of Science ®Google Scholar
• Michiels F. M., Chappuis S., Caillou B., Pasini A., Talbot M., Monier R., Lenoir G. M., Feunteun J., Billaud M. Development of medullary thyroid carcinoma in transgenic mice expressing the ret protooncogene altered by a multiple endocrine neoplasia type 2a mutation. Proceedings of The National Academy of Sciences of the United States of America. 1997; 94: 3330–3335
   PubMed Web of Science ®Google Scholar
• Moore M. W., Klein R. D., Farinas I., Sauer H., Armanini M., Phillips H., Reichardt L. F., Ryan A. M., Carver Moore K., Rosenthal A. Renal and neuronal abnormalities in mice lacking GDNF. Nature 1996; 382: 76–79
   PubMed Web of Science ®Google Scholar
• Moskaluk C. A., Kern SE. Cancer gets Mad: DPC4 and other TGFbeta pathway genes in human cancer. Biochim Biophys Acta 1996; 1288: M31–M33
   Google Scholar
• Mucke L., Oldstone M. B., Morris J. C., Nerenberg MI. Rapid activation of astrocyte-specific expression of GFAP-lacZ transgene by focal injury. New Biol 1991; 3: 465–474
   Google Scholar
• Mulligan L. M., Eng C., Healey C. S., Clayton D., Kwok J. B., Gardner E., Ponder M. A., Frilling A., Jackson C. E., Lehnert H., et al. Specific mutations of the RET proto-oncogene are related to disease phentype in MEN 2A and FMTC. Nat Genet 1994a; 6: 70–74
   PubMed Web of Science ®Google Scholar
• Mulligan L. M., Eng C., Healey C. S., Ponder M. A., Feldman G. L., Li P., Jackson C. E., Ponder BA. A de novo mutation of the RET proto-oncogene in a patient with MEN 2A. Hum Mol Genet 1994b; 3: 1007–1008
   Google Scholar
• Myers S. M., Eng C., Ponder B. A., Mulligan LM. Characterization of RET proto-oncogene 3′ splicing variants and polyadenylation sites: a novel C-terminus for RET. Oncogene 1995; 11: 2039–2045
   PubMed Web of Science ®Google Scholar
• No D., Yao T. P., Evans RM. Ecdysone-inducible gene expression in mammalian cells and transgenic mice. Proc Natl Acad Sci USA 1996; 93: 3346–3351
   PubMed Web of Science ®Google Scholar
• Norimura T., Nomoto S., Katsuki M., Gondo Y., Kondo S. p53-dependent apoptosis suppresses radiation-induced teratogenesis [see comments]. Nat Med 1996; 2: 577–580
   PubMed Web of Science ®Google Scholar
• Oro A. E., Higgins K. M., Hu Z. L., Bonifas J. M., Epstein E. H., Scott MP. Basal cell carcinomas in mice overexpressing sonic hedgehog. Science 1997; 276: 817–821
   PubMed Web of Science ®Google Scholar
• Palmiter R. D., Chen H. Y., Messing A., Brinster RL. SV40 enhancer and large T antigen are instrumental in development of choroid plexus tumours in trnsgenic mice. Nature 1985; 316: 457–460
   PubMed Web of Science ®Google Scholar
• Pandit S. D., Donis Keller H., Iwamoto T., Tomich J. M., Pike LJ. The multiple endocrine neoplasia type 2B point mutation alters long-term regulation and enhances the transforming capacity of the epidermal growth factor receptor. J Biol Chem 1996; 271: 5850–5858
   PubMedGoogle Scholar
• Pasini B., Borrello M. G., Greco A., Bongarzone I., Luo Y., Mondellini P., Alberti L., Miranda C., Arighi E., Bocciardi R, et al. Loss of function effect of RET mutations causing Hirschsprung disease. Nat Genet 1995; 10: 35–40
   PubMed Web of Science ®Google Scholar
• Peterson K. R., Clegg C. H., Huxley C., Josephson B. M., Haugen H. S., Furukawa T., Stamatoyannopoulos G. Transgenic mice containing a 248 kb yeast artificial chromosome carrying the human beta-globin locus display proper developmental control of human globin genes. Proc Natl Acad Sci USA 1993; 90: 7593–7597
   PubMed Web of Science ®Google Scholar
• Peterson K. R., Clegg C. H., Navas P. A., Norton E. J., Kimbrough T. G., Stamatoyannopoulos G. Effect of deletion of 5′HS3 or 5′HS2 of the human beta-globin locus control region on the developmental regulation of globin gene expression in berta-globin locus yeast artificial chromosome transgenic mice. Proc Natl Acad Sci USA 1996; 93: 6605–6609
   PubMedGoogle Scholar
• Peterson K. R., Li Q. L., Clegg C. H., Furukawa T., Navas P. A., Norton E. J., Kimbrough T. G., Stamatoyannopoulos G. Use of yeast artificial chromosomes (YACs) in studies of mammalian development: production of beta-globin locus YAC mice carrying human globin developmental mutants. Proc Natl Acad Sci USA 1995; 92: 5655–5659
   PubMed Web of Science ®Google Scholar
• Pichel J. G., Shen L., Sheng H. Z., Granholm A. C., Drago J., Grinberg A., Lee E. J., Huang S. P., Saarma M., Hoffer B. J., Sariola H., Westphal H. Defects in enteric innervation and kidney development in mice lacking GDNF. Nature 1996; 382: 73–76
   PubMed Web of Science ®Google Scholar
• Pietsch T., Waha A., Koch A., Kraus J., Albrecht S., Tonn J., Sorensen N., Berthold F., Henk B., Schmandt N., Wolf H. K., Von Deimling A., Wainwright B., Chenovix Trench G., Wiestler O. D., Wicking C. Medulloblastomas of the desmoplastic variant carry mutations of the human homologue of Drosophila patched. Cancer Res 1997; 57: 2085–2088
   PubMed Web of Science ®Google Scholar
• Plate K. H., Breier G., Millauer B., Ullrich A., Risau W. Up-regulation of vascular endothelial growth factor and its cognate receptors in a rat glioma model of tumor angiogenesis. Cancer Res 1993; 53: 5822–5827
   PubMed Web of Science ®Google Scholar
• Raffel C., Jenkins R. B., Frederick L., Hebrink D., Alderete B., Fults D. W., James C.D. Sporadic medullobalsto-mas contain PTCH mutations. Cancer Res 1997; 57: 842–845
   PubMed Web of Science ®Google Scholar
• Rickert R. C., Roes J., Rajewsky K. B lymphocyte-specific, Cre-mediated mutagenesis in mice. Nucleic Acids Res 1997; 25: 1317–1318
   PubMed Web of Science ®Google Scholar
• Roessler E., Belloni E., Gaudenz K., Jay P., Berta P., Scherer S. W., Tsui L. C., Muenke M. Mutations in the human Sonic Hedgehog gene cause holoprosence-phaly. Nat Genet 1996; 14: 357–360
   PubMed Web of Science ®Google Scholar
• Rovigatti U., Martucciello G., Jasonni V. Genetic alterations in neuroblastoma appear as a genetic make-up more than a single locus mutation. Heridi-tary Cancer 2, J-M Haefliger, H-J Muller, RJ Scott, W Weber, 1997; 11–36
   Google Scholar
• Rovigatti U. Isolation and initial characterization of a new virus: Micro-Foci inducing virus or MFV. CR Acad Sci III 1992; 315: 195–202
   Google Scholar
• Saenz, Robles M. T., Symonds H., Chen J., Van Dyke T. Induction versus progression of brain tumor development: differential functions for the pRB- and p53-targeting domains of simian virus 40 T antigen. Mol Cell Biol 1994; 14: 2686–2698
   Google Scholar
• Sagara M., Sugiyama F., Horiguchi H., Kamma H., Ogata T., Yagami K., Murakami K., Fukamizu A. Activation of the nuclear oncogenes N-myc and c-jun in carcinoid (correction of cartinoid) tumors of trans-genic mice carrying the human adenovirus type 12 El region gene. DNA Cell Biol 1995; 14: 95–101
   PubMedGoogle Scholar
• Sanchez M. P., Silos Santiago I., Frisen J., He B., Lira S. A., Barbacid M. Renal agenesis and the absence of enteric neurons in mice lacking GDNF. Nature 1996; 382: 70–73
   PubMed Web of Science ®Google Scholar
• Schedl A., Larin Z., Montoliu L., Thies E., Kelsey G., Lehrach H., Schutz G. A method for the generation of YAC transgenic mice by pronuclear microinjection. Nucleic Acids Res 1993; 21: 4783–4787
   PubMedGoogle Scholar
• Schuchardt A., D'Agati V., Larsson Blomberg L., Costantini F., Pachnis V. Defects in the kidney and enteric nervous system of mice lacking the tyrosine kinase receptor Ret (see comments). Nature 1994; 367: 380–383
   PubMed Web of Science ®Google Scholar
• Schuchardt A., D'Agati V., Larsson Blomberg L., Costantini F., Pachnis V. RET-deficient mice: an animal model for Hirschsprung's disease and renal agenesis. J Intern Med 1995; 238: 327–332
   PubMedGoogle Scholar
• Schuchardt A., D'Agati V., Pachnis V., Costantini F. Renal agenesis and hypodysplasia in ret-k- mutant mice result from defects in uretreric bud development. Development 1996; 122: 1919–1929
   PubMed Web of Science ®Google Scholar
• Servenius B., Vernachio J., Price J., Andersson L. C., Peterson PA. Metastasizing neuroblastomas in mice transgenic for simian virus 40 large T (SV40T) under the olfactory marker protein gene promoter. Cancer Res 1994; 54: 5198–5205
   Google Scholar
• Sharan S. K., Morimatsu M., Albrecht U., Lim D. S., Regel E., Dinh C., Sands A., Eichele G., Hasty P., Bradley A. Embryonic lethality and radiation hypersensi-tivity mediated by rad51 in mice lacking brca2. Nature 1997; 386: 804–810
   PubMed Web of Science ®Google Scholar
• Shimada H., Strain D. O., Chatten J., Joshi V. V., Hachitanda Y., Brodeur G. M., Lukens J. N., Matthay K. K., Seeger RC. Identification of subsets of neuroblastomas by combined histopathologic and N-myc analysis. J Natl Cancer Inst 1995; 87: 1470–1476
   PubMedGoogle Scholar
• Shockett P., Difilippantonio M., Hellman N., Schatz DG. A modified tetracycline-regulated system provides autoregulatory, inducible gene expression in cultured cells and transgenic mice. Proc Natl Acad Sci USA 1995; 92: 6522–6526
   PubMed Web of Science ®Google Scholar
• Small J. A., Blair D. G., Showalter S. D., Scangos GA. Analysis of a transgenic mouse containing simian virus 40 and v-myc sequences. Mol Cell Biol 1985; 5: 642–648
   PubMedGoogle Scholar
• Small J. A., Khoury G., Jay G., Howley P. M., Scangos GA. Early regions of JC virus and BK virus induce distinct and tissue-specific tumors in transgenic mice. Proc Natl Acad Sci USA 1986a; 83: 8288–8292
   PubMedGoogle Scholar
• Small J. A., Scangos G. A., Cork L., Jay G., Khoury G. The early region of human papovavirus JC induces dysmyelination in transgenic mice. Cell 1986b; 46: 13–18
   PubMedGoogle Scholar
• Son J. H., Chung J. H., Huh S. O., Park D. H., Peng C., Rosenblum M. G., Chung Y. I., Joh TH. Immortalization of neuroendocrine pinealocytes from transgenic mice by targeted tumorigenesis using the tryptophan hydro-xylase promoter. Brain Res Mol Brain Res 1996; 37: 32–40
   PubMedGoogle Scholar
• St Onge L., Furth P. A., Gruss P. Temporal control of the Cre recombinase in transgenic mice by a tetracycline responsive promoter. Nucleic Acids Res 1996; 24: 3875–3877
   Google Scholar
• Stone D. M., Hynes M., Armanini M., Swanson T. A., Gu Q., Johnson R. L., Scott M. P., Pennica D., Goddard A., Phillips H., Noll M., Hooper J. E., Sauvage De F., Rosenthal A. The tumour-suppressor gene patched encodes a candidate receptor for Sonic hedgehog (see comments). Nature 1996; 384: 129–134
   PubMed Web of Science ®Google Scholar
• Sugiyama F., Sagara M., Matsuda Y., Horiguchi H., Kamma H., Ogata T., Hatae T., Yagami K., Murakami K., Fukamizu A. Heritable formation of neuroecto-dermal tumor in transgenic mice carrying the combined El region gene of adenovirus type 12 with the deregulated human renin promoter. J Cell Biochem 1995; 57: 691–700
   Google Scholar
• Suzuki A., Delapompa J. L., Hakem R., Elia A., Yoshida R., Mo R., Nishina H., Chuang T., Wakeham A., Itie A., Koo W., Billia P., Ho A., Fukumoto M., Hui C. C., Mak TW. Brca2 is required for embryonic cellular proliferation in the mouse. Genes and Development 1997; 11: 1242–1252
   PubMed Web of Science ®Google Scholar
• Symonds H., Krall L., Remington L., Saenz Robles M., Lowe S., Jacks T., Van Dyke T. p53-dependent apoptosis suppresses tumor growth and progression. in vivo. Cell 1994; 78: 703–711
   Google Scholar
• The I., Hannigan G. E., Cowley G. S., Reginald S., Zhong Y., Gusella J. F., Hariharan I. K., Bernards A. Rescue of a drosophila nfl mutant phenotype by protein kinase a. Science 1997; 276: 791–794
   PubMedGoogle Scholar
• Treanor J. J., Goodman L., De Sauvage F., Stone D. M., Poulsen K. T., Beck C. D., Gray C., Armanini M. P., Pollock R. A., Hefti F., Phillips H. S., Goddard A., Moore M. W., Buj Bello A., Davies A. M., Asai N., Takahashi M., Vandlen R., Henderson C. E., Rosenthal A. Characterization of a multicomponent receptor for GDNF (see comments). Nature 1996; 382: 80–83
   PubMed Web of Science ®Google Scholar
• Trupp M., Arenas E., Fainzilber M., Nilsson A. S., Sieber B. A., Grigoriou M., Kilkenny C., Salazar Grueso E., Pachnis V., Arumae U, et al. Functional receptor for GDNF encoded by the c-ret proto-oncogene (see comments). Nature 1996; 381: 785–788
   PubMed Web of Science ®Google Scholar
• Tsuchida Y., Hemmi H., Inoue A., Obana K., Yang H. W., Hayashi Y., Kanda N., Shimatake H. Genetic clinical markers of human neuroblastoma with special reference to N-myc oncogene: amplified or not amplified?-An overview. Tumour Biol 1996; 17: 65–74
   PubMedGoogle Scholar
• Turgeon J. L., Kimura Y., Waring D. W., Mellon PL. Steroid and pulsatile gonadotropin-releasing hormone (GnRH) regulation of luteinizing hormone and GnRH receptor in a novel gonadotrope cell line. Mol Endocrinol 1996; 10: 439–450
   PubMedGoogle Scholar
• Viskochil D., Buchberg A. M., Xu G., Cawthon R. M., Stevens J., Wolff R. K., Culver M., Carey J. C., Copeland N. G., Jenkins NA, et al. Deletions and a translocation interrupt a cloned gene at the neurofibromatosis type 1 locus. Cell 1990; 62: 187–192
   PubMed Web of Science ®Google Scholar
• Vorechovsky I., Tingby O., Hartman M., Stromberg B., Nister M., Collins V. P., Toftgard R. Somatic mutations in the human homologue of drosophila patched in primitive neuroectodermal tumours. Onco-gene. 1997; 15: 361–366
   Google Scholar
• Wallace M. R., Marchuk D. A., Andersen L. B., Letcher R., Odeh H. M., Saulino A. M., Fountain J. W., Brereton A., Nicholson J., Mitchell A.L., et al. Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NFl patients [published erratum appears in. Science 1990; 249: 181–186, Science 250 :1749].
   PubMed Web of Science ®Google Scholar
• Weiss W. A., Aldape K., Mohapatra G., Feuerstein B. G., Bishop JM. Targeted expression of mycn causes neuroblastoma in transgenic mice. Ennbo Journal 1997; 16: 2985–2995
   PubMed Web of Science ®Google Scholar
• Weissenberger J., Steinbach J. P., Malin G., Spada S., Rulicke T., Aguzzi A. Development and malignant progression of astrocytomas in GFAP-v-src transgenic mice. Oncogene 1997; 14: 2005–2013
   PubMed Web of Science ®Google Scholar
• Weller M., Malipiero U., Aguzzi A., Reed J. C., Fontana A. Protooncogene bcl-2 gene transfer abrogates Fas/APO-1 antibody-mediated apoptosis of human malignant glioma cells and confers resistance to chemotherapeutic drugs and therapeutic irradiation. J Clin Invest 1995; 95: 2633–2643
   PubMed Web of Science ®Google Scholar
• Xie J., Johnson R. L., Zhang X., Bare J. W., Waldman F. M., Cogen P. H., Menon A. G., Warren R. S., Chen L. C., Scott M. P., Epstein E.H., Jr. Mutations of the PATCHED gene in several types of sporadic extracutaneous tumors. Cancer Res 1997; 57: 2369–2372
   PubMed Web of Science ®Google Scholar
• Xu G. F., O'Connell P., Viskochil D., Cawthorn R., Robertson M., Culver M., Dunn D., Stevens J., Gesteland R., White R., et al. The neurofibromatosis type 1 gene encodes a protein related to GAP. Cell 1990; 62: 599–608
   PubMed Web of Science ®Google Scholar
• Yahanda A. M., Bruner J. M., Donehower L. A., Morrison RS. Astrocytes derived from p53-deficient mice provide a multistep in vitro model for development of malignant gliomas. Mol Cell Biol 1995; 15: 4249–4259
   PubMedGoogle Scholar
• Yin C., Knudson C. M., Korsmeyer S. J., Van Dyke T. Bax suppresses tumorigenesis and stimulates apoptosis. in vivo. Nature 1997; 385: 637–640
   PubMed Web of Science ®Google Scholar
• Zacksenhaus E., Jiang Z., Chung D., Marth J. D., Phillips R. A., Gaille BL. pRb controls proliferation, differentiation, and death of skeletal muscle cells and other lineages during embryogenesis. Genes Dev 1996; 10: 3051–3064
   PubMed Web of Science ®Google Scholar
• Zou Y. R., Muller W., Gu H., Rajewsku K. Cre-loxP-mediated gene replacement: a mouse strain producing humanized antibodies. Curr Biol 1994; 4: 1099–1103
   Google Scholar