Structural Basis of Merlin Tumor Suppressor Functions in Neurofibromatosis-2
Abstract
Loss-of-function mutations in the neurofibromatosis-2 (NF2) gene lead to familial and sporadic neurological malignancies in man, specifically to schwannomas and meningiomas, and also to malignant mesothelioma in the lung. Furthermore, Nf2 heterozygosity in mice leads to the development of a number of aggressive tumors, indicating that merlin, the cytoskeletal protein encoded by the NF2 gene, plays broad roles in harnessing tumorigenesis, which are essentially incurable and the overall outcome of Neurofibromatosis Type II (NF2) patients with these cancers remains dismal, despite aggressive therapy.
Document Details
- Document Type
- Technical Report
- Publication Date
- Oct 01, 2013
- Accession Number
- ADA593130
Entities
People
- Tina Izard
Organizations
- Scripps Research