- References (2)
- Inventor Info
|Antigen/Gene or Protein Targets||Ptch (Protein Patched Homolog)|
|Disease Keywords||Gastro Intestinal Stromal Tumour|
|Relevance||PTCH1 is a member of the patched gene family and is the receptor for sonic hedgehog, a secreted molecule implicated in the formation of embryonic structures and in tumorigenesis. The aberration of the Hedgehog signalling in monocytes (LysM positive cells) induces Pdgfr alpha-positive, Kit-negative GIST(GastroIntestinal Stromal Tumour)-like tumours. This model is a very potent tool for the screening of potential new therapeuticsfor GIST, especially within the Hedgehog signalling.|
|Production Details||Ptch flox/flox mice have loxP sites in Ptch introns 7 and 924. LysMcre mice express Cre recombinase under the control of the endogenous LysM-promoter. This Cre driver targets mouse cells of the myeloid lineage, due to their expression of lysozyme M.|
|Breeding Information||Good and effective breeding.|
|Conditional Description||Mouse Ptch is conditionally inactivated in monocytes using a Cre recombinase driven by the lysozyme M (LysM) promoter.|
|Growth/Phenotype Keywords||Severe gastrointestinal phenotype without affecting otherwise normal development.|
|Mouse Genetic Background/Cross History||C57BL/6 x Balb/c (PtchF/F) x C57BL/6 (LysMCre)|
|Zygosity||Ptch Flox/Flox-LysM Cre+/-|
|Research Area||Cancer, Cell Cycle, Cell Signaling & Signal Transduction, Genetic Studies Tools, Immunology, Neurobiology|
This is the first immunological GIST mouse model with an aberrant Hedgehog signaling independent of Kit, demonstrating the importance of this particular pathway in GIST formation.
Proof of concept with Imatinib treatment.
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