Abstract
Basal cell carcinomas (BCCs) were essentially a molecular 'black box' until some 12 years ago, when identification of a genetic flaw in a rare subset of patients who have a great propensity to develop BCCs pointed to aberrant Hedgehog signalling as the pivotal defect leading to formation of these tumours. This discovery has facilitated a remarkable increase in our understanding of BCC carcinogenesis and has highlighted the carcinogenic role of this developmental pathway when aberrantly activated in adulthood. Importantly, a phase 1 first-in-human trial of a Hedgehog inhibitor has shown real progress in halting and even reversing the growth of these tumours.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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Antineoplastic Agents / therapeutic use
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Basal Cell Nevus Syndrome / genetics
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Basal Cell Nevus Syndrome / physiopathology
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Carcinoma, Basal Cell / drug therapy
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Carcinoma, Basal Cell / epidemiology
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Carcinoma, Basal Cell / genetics
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Carcinoma, Basal Cell / metabolism*
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Genetic Predisposition to Disease
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Hedgehog Proteins / antagonists & inhibitors
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Hedgehog Proteins / genetics
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Hedgehog Proteins / metabolism*
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Humans
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Mice
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Patched Receptors
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Receptors, Cell Surface / genetics
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Signal Transduction / drug effects
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Skin Neoplasms / drug therapy
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Skin Neoplasms / epidemiology
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Skin Neoplasms / genetics
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Skin Neoplasms / metabolism*
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United States / epidemiology
Substances
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Antineoplastic Agents
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Hedgehog Proteins
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Patched Receptors
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Receptors, Cell Surface