NF1 tumor suppressor protein and mRNA in skeletal tissues of developing and adult normal mouse and NF1-deficient embryos

被引:60
作者
Kuorilehto, T
Nissinen, M
Koivunen, J
Benson, MD
Peltonen, J
机构
[1] Univ Oulu, Dept Anat & Cell Biol, FIN-90014 Oulu, Finland
[2] Univ Texas, SW Med Ctr, Ctr Dev Biol, Dallas, TX USA
[3] Univ Oulu, Dept Dermatol, Oulu, Finland
[4] Univ Turku, Dept Med Biochem, Turku, Finland
关键词
neurofibromatosis; neurofibromin; cartilage; bone; Ras;
D O I
10.1359/JBMR.040130
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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. The NF1 gene was expressed intensely in the cartilage and the periosteum. Impaired NF1 expression may lead to inappropriate development and dynamics of bones and ultimately to the osseous manifestations of the disease.
引用
收藏
页码:983 / 989
页数:7
相关论文
共 39 条
  • [1] ABERRANT REGULATION OF RAS PROTEINS IN MALIGNANT-TUMOR CELLS FROM TYPE-1 NEUROFIBROMATOSIS PATIENTS
    BASU, TN
    GUTMANN, DH
    FLETCHER, JA
    GLOVER, TW
    COLLINS, FS
    DOWNWARD, J
    [J]. NATURE, 1992, 356 (6371) : 713 - 715
  • [2] TARGETED DISRUPTION OF THE NEUROFIBROMATOSIS TYPE-1 GENE LEADS TO DEVELOPMENTAL ABNORMALITIES IN HEART AND VARIOUS NEURAL CREST-DERIVED TISSUES
    BRANNAN, CI
    PERKINS, AS
    VOGEL, KS
    RATNER, N
    NORDLUND, ML
    REID, SW
    BUCHBERG, AM
    JENKINS, NA
    PARADA, LF
    COPELAND, NG
    [J]. GENES & DEVELOPMENT, 1994, 8 (09) : 1019 - 1029
  • [3] CHONDROCYTE DIFFERENTIATION
    CANCEDDA, R
    CANCEDDA, FD
    CASTAGNOLA, P
    [J]. INTERNATIONAL REVIEW OF CYTOLOGY - A SURVEY OF CELL BIOLOGY, VOL 159, 1995, 159 : 265 - 358
  • [4] IDENTIFICATION AND CHARACTERIZATION OF TRANSCRIPTS FROM THE NEUROFIBROMATOSIS-1 REGION - THE SEQUENCE AND GENOMIC STRUCTURE OF EVI2 AND MAPPING OF OTHER TRANSCRIPTS
    CAWTHON, RM
    OCONNELL, P
    BUCHBERG, AM
    VISKOCHIL, D
    WEISS, RB
    CULVER, M
    STEVENS, J
    JENKINS, NA
    COPELAND, NG
    WHITE, R
    [J]. GENOMICS, 1990, 7 (04) : 555 - 565
  • [5] NF1 tumor suppressor gene function: Narrowing the GAP
    Cichowski, K
    Jacks, T
    [J]. CELL, 2001, 104 (04) : 593 - 604
  • [6] OSSEOUS MANIFESTATIONS OF NEUROFIBROMATOSIS IN CHILDHOOD
    CRAWFORD, AH
    BAGAMERY, N
    [J]. JOURNAL OF PEDIATRIC ORTHOPAEDICS, 1986, 6 (01) : 72 - 88
  • [7] THE PROTEIN PRODUCT OF THE NEUROFIBROMATOSIS TYPE-1 GENE IS EXPRESSED AT HIGHEST ABUNDANCE IN NEURONS, SCHWANN-CELLS, AND OLIGODENDROCYTES
    DASTON, MM
    SCRABLE, H
    NORDLUND, M
    STURBAUM, AK
    NISSEN, LM
    RATNER, N
    [J]. NEURON, 1992, 8 (03) : 415 - 428
  • [8] NEUROFIBROMIN, A PREDOMINANTLY NEURONAL GTPASE ACTIVATING PROTEIN IN THE ADULT, IS UBIQUITOUSLY EXPRESSED DURING DEVELOPMENT
    DASTON, MM
    RATNER, N
    [J]. DEVELOPMENTAL DYNAMICS, 1992, 195 (03) : 216 - 226
  • [9] Targeting ras signalling pathways in cancer therapy
    Downward, J
    [J]. NATURE REVIEWS CANCER, 2003, 3 (01) : 11 - 22
  • [10] Friedman JM, 1997, AM J MED GENET, V70, P138, DOI 10.1002/(SICI)1096-8628(19970516)70:2<138::AID-AJMG7>3.0.CO