Abstract:
:The variable manifestation of phenotypes that occur in patients with neurofibromatosis type 1 (NF1) includes benign and malignant neurocutaneous tumors for which no adequate treatment exists. Cell-based screening of known bioactive compounds library identified the protein phosphatase 2A (PP2A) inhibitor Cantharidin and the L-type calcium channel blocker Nifedipine as potential candidates for NF1 pharmacotherapy. Validation of screening results using human NF1-associated malignant peripheral nerve sheath tumor (MPNST) cells showed that Cantharidin effectively impeded MPNST cell growth, while Nifedipine treatment significantly decreased local tumor growth in an MPNST xenograft animal model. These data suggest that inhibitors of PP2A, as well as calcium channel blockers, might be used in broader MPNST preclinical studies as single agents or in combinatorial therapeutic strategies.
journal_name
Biochimiejournal_title
Biochimieauthors
Semenova G,Stepanova DS,Deyev SM,Chernoff Jdoi
10.1016/j.biochi.2017.01.001subject
Has Abstractpub_date
2017-04-01 00:00:00pages
1-5eissn
0300-9084issn
1638-6183pii
S0300-9084(17)30001-9journal_volume
135pub_type
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