caa a22 4500 475845447 CHVBK 20180406123912.0 cr unu---uuuuu 170329e20001101xx s 000 0 eng 10.1007/PL00000654 doi (NATIONALLICENCE)springer-10.1007/PL00000654 Toftgård R. Karolinska Institutet, Department of Biosciences and Center for Nutrition and Toxicology, Novum, SE-141 57 Huddinge (Sweden), Fax +46 8 6081501, e-mail: rune.toftgard@cnt.ki.se, SE aut Hedgehog signalling in cancer [Elektronische Daten] [R. Toftgård] Abstract.: Hedgehog signalling is a key regulator of embryonic development controlling proliferation and/or cell fate determination. With identification of the Hedgehog receptor PTCH1 as a tumour suppressor gene that underlies the human nevoid basal cell carcinoma syndrome (NBCCS), the Hedgehog signalling pathway was firmly linked to cancer. It now appears that constitutive activation of Hedgehog signalling, by inactivating mutations in PTCH1 or activating mutations in the coreceptor SMOH, is required and possibly sufficient for basal cell carcinoma development and also contributes to the formation of a variety of other tumour types, including medulloblastoma and rhabdomyosarcoma. Several lines of evidence, including transgenic mice experiments, suggest that the critical cellular effect is stimulation of proliferation mediated by the transcriptional effector GLI1. Additional components of the signal transduction machinery as well as essential target genes remain to be identified, and involvement of the Hedgehog signalling pathway in other tumour types and/or hereditary cancer predisposition syndromes is to be expected. Birkhäuser Verlag Basel,, 2000 Key words. Hedgehog signalling nationallicence PTCH tumour suppressor nationallicence GLI1 nationallicence suppressor of fused nationallicence basal cell cancer nationallicence medulloblastoma nationallicence cancer nationallicence https://doi.org/10.1007/PL00000654 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00000654 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Toftgård R. Karolinska Institutet, Department of Biosciences and Center for Nutrition and Toxicology, Novum, SE-141 57 Huddinge (Sweden), Fax +46 8 6081501, e-mail: rune.toftgard@cnt.ki.se, SE aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer