Table 3Mouse models of Hh-induced skin tumorigenesis. Additional table references: 207-229

Gene TissueTransgene/MutantHh StatusEffectRefs.
Shh Basal K14-Shh epitheliaElevated Hh ligand, overwhelms negative regulationEmbryonic BCC-like tumors; perinatal lethality limits tumor studies96
Ptc1 Global Ptc1lac/+ Decreased negative regulation of Hh responsesLow frequency spontaneous trichoblastomas and BCCs; Increased induction of BCCs and rare SCCs on irradiation treatment5
Ptc2 Global Ptc2TM1/TM1 Decreased negative regulation of Hh responsesMale-specific alopecia and ulcered lesions develop with ageE.N. and C.c.H., unpubl.
Smo Basal epithelia K5-M2SMO Ligand-independent activation of Hh responsesBCC-like proliferations in neonatal skin; perinatal lethality limits tumor studies148
Basal epithelia ΔK5-M2SMO Ligand-independent activation of Hh responsesBenign basaloid follicular hamartomas146
Gli1 Basal epithelia K5-Gli1 Unregulated GliACTSpontaneous BCC-like tumors147
Basal epithelia K14-Gli1 Unregulated GliACT, may be subject to endogenous Hh signalHF tumors during anagen100
Gli2 Basal epithelia K5-Gli2 Increased Gli species, subject to endogenous Hh signalsSpontaneous BCC and basaloid follicular hamartomas; slower, milder tumor phenotype than Gli1 or DNGli2 overexpression145, 146
Basal epithelia K5-Cre;Z/AP-Gli2 Increased Gli species, subject to endogenous Hh signalsSpontaneous BCC and basaloid follicular hamartomas; slower, milder tumor phenotype than Gli1 or DNGli2 overexpressionP.M. and C.c.H., unpubl.
Basal epithelia K5-ΔNGli2 Unregulated GliACTSpectrum of HF-derived tumors (BCCs, trichoblastomas, cylindroma, trichoepithelioma) ; rapid, aggressive tumor phenotype162
Basal epithelia K5-Cre;Z/AP-ΔNGli2 Unregulated GliACTSpectrum of HF-derived tumors (BCCs, trichoblastomas, cylindroma, trichoepithelioma) ; rapid, aggressive tumor phenotypeP.M. and C.c.H., unpubl.
Basal epithelia K5-tTA;TRE-Gli2 Inducible elevated Gli species, subject to endogenous HhSpontaneous BCCs requiring continuous Hh signaling for growth, transgene inactivation results in tumor regression144

From: Splitting Hairs: Dissecting the Roles of Gli Activator and Repressor Functions during Epidermal Development and Disease

Cover of Madame Curie Bioscience Database
Madame Curie Bioscience Database [Internet].
Austin (TX): Landes Bioscience; 2000-2013.
Copyright © 2000-2013, Landes Bioscience.

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.