Article
A murine model of neurofibromatosis type 2 that accurately phenocopies human schwannoma formation.
Human molecular genetics - 1 Jan 2015
Gehlhausen Jeffrey R, Park Su-Jung, Hickox Ann E, Shew Matthew, Staser Karl, Rhodes Steven D, Menon Keshav, Lajiness Jacquelyn D, Mwanthi Muithi, Yang Xianlin, Yuan Jin, Territo Paul, Hutchins Gary, Nalepa Grzegorz, Yang Feng-Chun, Conway Simon J, Heinz Michael G, Stemmer-Rachamimov Anat, Yates Charles W, Wade Clapp D
Abstract excerpt
Neurofibromatosis type 2 (NF2) is an autosomal dominant genetic disorder resulting from germline mutations in the NF2 gene. Bilateral vestibular schwannomas, tumors on cranial nerve VIII, are pathognomonic for NF2 disease. Furthermore, schwannomas also commonly develop in other cranial nerves, dorsal root ganglia and peripheral nerves. These tumors are a major cause of morbidity and mortality, and medical...
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