About: Sixteen Years and Counting: The Current Understanding of Fibroblast Growth Factor Receptor 3 (FGFR3) Signaling in Skeletal Dysplasias     Goto   Sponge   NotDistinct   Permalink

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  • In 1994, the field of bone biology was significantly advanced by the discovery that activating mutations in the fibroblast growth factor receptor 3 (FGFR3) receptor tyrosine kinase (TK) account for the common genetic form of dwarfism in humans, achondroplasia (ACH). Other conditions soon followed, with the list of human disorders caused by FGFR3 mutations now reaching at least 10. An array of vastly different diagnoses is caused by similar mutations in FGFR3. As FGFR3-related skeletal dysplasias are caused by growth attenuation of the cartilage, chondrocytes appear to be unique in their response to FGFR3 activation. The full spectrum of molecular events by which FGFR3 mediates its signaling is just beginning to emerge. This article describes the challenging journey to unravel the mechanisms of FGFR3 function in skeletal dysplasias, the extraordinary cellular manifestations of FGFR3 signaling in chondrocytes, and finally, the progress toward therapy for ACH and cancer.
  • In 1994, the field of bone biology was significantly advanced by the discovery that activating mutations in the fibroblast growth factor receptor 3 (FGFR3) receptor tyrosine kinase (TK) account for the common genetic form of dwarfism in humans, achondroplasia (ACH). Other conditions soon followed, with the list of human disorders caused by FGFR3 mutations now reaching at least 10. An array of vastly different diagnoses is caused by similar mutations in FGFR3. As FGFR3-related skeletal dysplasias are caused by growth attenuation of the cartilage, chondrocytes appear to be unique in their response to FGFR3 activation. The full spectrum of molecular events by which FGFR3 mediates its signaling is just beginning to emerge. This article describes the challenging journey to unravel the mechanisms of FGFR3 function in skeletal dysplasias, the extraordinary cellular manifestations of FGFR3 signaling in chondrocytes, and finally, the progress toward therapy for ACH and cancer. (en)
Title
  • Sixteen Years and Counting: The Current Understanding of Fibroblast Growth Factor Receptor 3 (FGFR3) Signaling in Skeletal Dysplasias
  • Sixteen Years and Counting: The Current Understanding of Fibroblast Growth Factor Receptor 3 (FGFR3) Signaling in Skeletal Dysplasias (en)
skos:prefLabel
  • Sixteen Years and Counting: The Current Understanding of Fibroblast Growth Factor Receptor 3 (FGFR3) Signaling in Skeletal Dysplasias
  • Sixteen Years and Counting: The Current Understanding of Fibroblast Growth Factor Receptor 3 (FGFR3) Signaling in Skeletal Dysplasias (en)
skos:notation
  • RIV/68081707:_____/12:00375985!RIV13-AV0-68081707
http://linked.open...avai/predkladatel
http://linked.open...avai/riv/aktivita
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  • P(GA301/09/0587), P(GAP305/11/0752), S, Z(AV0Z50040507), Z(AV0Z60220518), Z(MSM0021622430)
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  • 1
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  • 168206
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  • RIV/68081707:_____/12:00375985
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  • FGFR3; chondrocyte; skeletal dysplasia; MAP kinase; FGF (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • US - Spojené státy americké
http://linked.open...ontrolniKodProRIV
  • [4303B15A8E00]
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  • Human Mutation
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  • 33
http://linked.open...iv/tvurceVysledku
  • Krejčí, Pavel
  • Foldynová-Trantírková, Silvie
  • Wilcox, W. R.
http://linked.open...ain/vavai/riv/wos
  • 000300705300005
http://linked.open...n/vavai/riv/zamer
issn
  • 1059-7794
number of pages
http://bibframe.org/vocab/doi
  • 10.1002/humu.21636
is http://linked.open...avai/riv/vysledek of
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