Document Detail

Hereditary tumor syndromes and gliomas.
MedLine Citation:
PMID:  19322539     Owner:  NLM     Status:  MEDLINE    
Several congenital syndromes caused by germline mutations in tumor suppressor genes predispose to the development of glial tumors. In the last few decades our knowledge about the molecular functions of these genes and the pathogenesis of hereditary tumor syndromes has greatly increased. The most common syndromes are the neurofibromatoses (type 1 and type 2) and the tuberous scleroses complex. There are interesting overlaps in the molecular pathogen-esis. Deregulation of Ras or downstream Ras pathways including MEK/ERK and AKT/ mTOR plays an important role in these three syndromes. Other rare syndromes include Li-Fraumeni, melanoma-astrocytoma, and Turcot syndrome involving cell cycle regulators and DNA repair genes. The genes and pathways involved in the pathogenesis of these syndromes also play an important role in the development of sporadic tumors. Therefore research on hereditary syndromes contributes substantially to our understanding of tumor formation.
David Reuss; Andreas von Deimling
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Recent results in cancer research. Fortschritte der Krebsforschung. Progrès dans les recherches sur le cancer     Volume:  171     ISSN:  0080-0015     ISO Abbreviation:  Recent Results Cancer Res.     Publication Date:  2009  
Date Detail:
Created Date:  2009-03-26     Completed Date:  2009-06-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0044671     Medline TA:  Recent Results Cancer Res     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  83-102     Citation Subset:  IM    
Department of Neuropathology, Institute of Pathology, Im Neuenheimer Feld 220/221, Heidelberg 69120, Germany.
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MeSH Terms
Brain Neoplasms / genetics*
Glioma / genetics*
Li-Fraumeni Syndrome / genetics
Melanoma / genetics
Neoplastic Syndromes, Hereditary / genetics*
Neurofibromatoses / genetics
Tuberous Sclerosis / genetics

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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