Establish an in vitro Model fo rthe Study of NF2 Gene Function and Gene Therapy
Abstract
Mutation analyses of the NF2 gene from NF2 patients suggest that the inactivation of the NF2 gene, and the consequent lack of gene product (protein known as Merlin/Schwannomin) is the primary cause of this disease. Within the three years period, we plan to develop a laboratory protocol to establish Schwann cell culture using surgical specimens from NF2 patients, and compare the tumor cells from patients with different NF2 gene mutations. In addition, we want to extend the life span of primary culture cells by immortalizing them using a retrovirus which we engineered. This will not only ensure the reproducibility of results within the NF2 community, but also provide scientists greater access to certain materials for the study of biologic function of Schwann cell and also important for testing therapeutic approaches. During the first 12 months, an additional 4 normal Schwann cells cultures were added to the previously banked cultures. One addition of primary human vestibular schwannoma culture from a NF2 patients and one normal Schwann cell culture are in the process of immortalization.
Document Details
- Document Type
- Technical Report
- Publication Date
- Oct 01, 2001
- Accession Number
- ADA400467
Entities
People
- Gene Hung
Organizations
- House Ear Institute