Molecular targets for emerging anti-tumor therapies for neurofibromatosis type 1.
Biochem Pharmacol
; 72(11): 1485-92, 2006 Nov 30.
Article
in En
| MEDLINE
| ID: mdl-16797490
ABSTRACT
Neurofibromatosis type 1 (NF1) is the most common cancer predisposition syndrome. NF1 patients present with a constellation of clinical manifestations and have an increased risk of developing certain benign and malignant tumors. This disease results from mutation within the gene encoding neurofibromin, a GTPase activating protein (GAP) for Ras. Functional loss of this protein compromises Ras inactivation, which leads to the aberrant growth and proliferation of neural crest-derived cells and, ultimately, tumor formation. Current management of NF1-associated malignancy involves radiation, surgical excision, and cytotoxic drugs. The limited success of these strategies has fueled researchers to further elucidate the molecular changes that drive tumor formation and progression. This discussion will highlight how intracellular signaling molecules, cell-surface receptors, and the tumor microenvironment constitute potential therapeutic targets, which may be relevant not only to NF1-related malignancy but also to other human cancers.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Genetic Therapy
/
Genes, Neurofibromatosis 1
/
Neurofibromatosis 1
/
Genetic Predisposition to Disease
/
Neurofibromin 1
Limits:
Animals
/
Humans
Language:
En
Journal:
Biochem Pharmacol
Year:
2006
Document type:
Article
Affiliation country: