Abstract
In this study we show in mice that Ftm (Rpgrip1l) is located at the ciliary basal body. Our data reveal that Ftm is necessary for developmental processes such as the establishment of left-right asymmetry and patterning of the neural tube and the limbs. The loss of Ftm affects the ratio of Gli3 activator to Gli3 repressor, suggesting an involvement of Ftm in Shh signalling. As Ftm is not essential for cilia assembly but for full Shh response, Ftm can be considered as a novel component for cilium-related Hh signalling. Furthermore, the absence of Ftm in arthropods underlines the divergence between vertebrate and Drosophila Hh pathways.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Adaptor Proteins, Signal Transducing / chemistry
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Adaptor Proteins, Signal Transducing / physiology*
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Animals
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Body Patterning / physiology*
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Cells, Cultured
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Cilia / metabolism*
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Embryo, Mammalian / metabolism
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Extremities / embryology
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Extremities / growth & development
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Hedgehog Proteins / metabolism*
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Kruppel-Like Transcription Factors / metabolism
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Mice
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Mice, Knockout
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Mutation
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Nerve Tissue Proteins / metabolism
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Neural Tube Defects / genetics
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Neural Tube Defects / metabolism
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Phenotype
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Polydactyly / genetics
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Polydactyly / metabolism
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Signal Transduction*
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Zinc Finger Protein Gli3
Substances
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Adaptor Proteins, Signal Transducing
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Ftm protein, mouse
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Gli3 protein, mouse
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Hedgehog Proteins
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Kruppel-Like Transcription Factors
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Nerve Tissue Proteins
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Shh protein, mouse
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Zinc Finger Protein Gli3