Document Detail

Nevoid basal cell carcinoma syndrome: molecular biology and new hypotheses.
MedLine Citation:
PMID:  10355946     Owner:  NLM     Status:  MEDLINE    
To understand the molecular biology of the nevoid basal cell carcinoma syndrome, the hedgehog signaling network is explained. The syndrome is caused by mutations in patched, a tumor suppressor gene. A single point mutation in one patched allele may be responsible for the malformations found in the syndrome. Inactivation of both patched alleles results in the formation of tumors and cysts (basal cell carcinomas, odontogenic keratocysts, and medulloblastomas). Several new hypotheses are proposed to account for a minority of nevoid basal cell carcinoma syndrome patients. Evidence is provided to suggest that these hypotheses deserve to be investigated.
M M Cohen
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  International journal of oral and maxillofacial surgery     Volume:  28     ISSN:  0901-5027     ISO Abbreviation:  Int J Oral Maxillofac Surg     Publication Date:  1999 Jun 
Date Detail:
Created Date:  1999-06-24     Completed Date:  1999-06-24     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8605826     Medline TA:  Int J Oral Maxillofac Surg     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  216-23     Citation Subset:  D; IM    
Department of Oral & Maxillofacial Pathology, Dalhousie University, Halifax, Nova Scotia, Canada.
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MeSH Terms
Basal Cell Nevus Syndrome / genetics*
Body Patterning / genetics
Embryonic Induction / genetics
Gene Expression Regulation, Developmental
Gene Expression Regulation, Neoplastic*
Genes, Tumor Suppressor / genetics
Hedgehog Proteins
Loss of Heterozygosity
Membrane Proteins / genetics,  physiology
Oncogene Proteins / genetics,  physiology
Point Mutation
Proteins / genetics,  physiology
Receptors, Cell Surface / genetics,  physiology
Receptors, G-Protein-Coupled*
Signal Transduction / genetics
Transcription Factors / genetics,  physiology
Reg. No./Substance:
0/Gli protein; 0/Hedgehog Proteins; 0/Membrane Proteins; 0/Oncogene Proteins; 0/Proteins; 0/Receptors, Cell Surface; 0/Receptors, G-Protein-Coupled; 0/SMO protein, human; 0/Trans-Activators; 0/Transcription Factors; 0/patched receptors

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