PMID- 22550417
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20120823
LR  - 20211021
IS  - 1369-1643 (Electronic)
IS  - 1357-714X (Print)
IS  - 1357-714X (Linking)
VI  - 2012
DP  - 2012
TI  - Calcitriol inhibits hedgehog signaling and induces vitamin d receptor signaling 
      and differentiation in the patched mouse model of embryonal rhabdomyosarcoma.
PG  - 357040
LID - 10.1155/2012/357040 [doi]
LID - 357040
AB  - Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. 
      Aberrant Hedgehog (Hh) signaling is characteristic of the embryonal subtype 
      (ERMS) and of fusion-negative alveolar RMS. In the mouse, ERMS-like tumors can be 
      induced by mutations in the Hh receptor Patched1 (Ptch). As in humans these 
      tumors show increased Hh pathway activity. Here we demonstrate that the treatment 
      with the active form of vitamin D(3), calcitriol, inhibits Hh signaling and 
      proliferation of murine ERMS in vivo and in vitro. Concomitantly, calcitriol 
      activates vitamin D receptor (Vdr) signaling and induces tumor differentiation. 
      In addition, calcitriol inhibits ERMS growth in Ptch-mutant mice, which is, 
      however, a rather late response. Taken together, our results suggest that 
      exogenous supply of calcitriol could be beneficial in the treatment of RMS, 
      especially in those which are associated with aberrant Hh signaling activity.
FAU - Uhmann, Anja
AU  - Uhmann A
AD  - Institute of Human Genetics, University of Goettingen, 37073 Gottingen, Germany.
FAU - Niemann, Hannah
AU  - Niemann H
FAU - Lammering, Berenice
AU  - Lammering B
FAU - Henkel, Cornelia
AU  - Henkel C
FAU - Hess, Ina
AU  - Hess I
FAU - Rosenberger, Albert
AU  - Rosenberger A
FAU - Dullin, Christian
AU  - Dullin C
FAU - Schraepler, Anke
AU  - Schraepler A
FAU - Schulz-Schaeffer, Walter
AU  - Schulz-Schaeffer W
FAU - Hahn, Heidi
AU  - Hahn H
LA  - eng
PT  - Journal Article
DEP - 20120221
PL  - Egypt
TA  - Sarcoma
JT  - Sarcoma
JID - 9709257
PMC - PMC3329653
EDAT- 2012/05/03 06:00
MHDA- 2012/05/03 06:01
PMCR- 2012/02/21
CRDT- 2012/05/03 06:00
PHST- 2011/09/28 00:00 [received]
PHST- 2011/12/07 00:00 [accepted]
PHST- 2012/05/03 06:00 [entrez]
PHST- 2012/05/03 06:00 [pubmed]
PHST- 2012/05/03 06:01 [medline]
PHST- 2012/02/21 00:00 [pmc-release]
AID - 10.1155/2012/357040 [doi]
PST - ppublish
SO  - Sarcoma. 2012;2012:357040. doi: 10.1155/2012/357040. Epub 2012 Feb 21.