Accession Number:

ADA460469

Title:

Biochemical Characterization of Native Schwannonmin/Merlin

Descriptive Note:

Annual summary rept. 11 Aug 2005-10 Aug 2006

Corporate Author:

ILLINOIS UNIV AT CHICAGO

Personal Author(s):

Report Date:

2006-09-01

Pagination or Media Count:

6.0

Abstract:

Neurofibromatosis type 2 NF2 is an autosomal dominant disorder characterized by the development of bilateral vestibular and spinal schwamomas meningiomas and ependymomas. The hF2 gene encodes a 595 amino acid polypeptide known as NF2 protein or Merlin or Schwanomin. The primary structure of the NF2 protein is homologous to the ERM family of peripheral membrane proteins which includes Eznn Radixin and Moesin. The founding member of the ERM superfamily is the erythrocyte membrane protein 4.1 which cross-links spectrin-actin complexes and attaches them to the plasma membrane. We have established that p55 a palmitoylated peripheral membrane phosphoprotein forms a ternary complex with protein 4.1 and glycophorin C. Notably the Drosophila homologue of pSS functions as a tumor suppressor in epithelial and neuronal tissues. In the 2nd year of the funding penod we demonstrated binding between pSS and the NF2 protein and established the existence of this complex in human erythrocyte plasma membrane. This unexpected finding revealed a new paradigm integrating the known functions of the pSS family of proteins with the pathophysiology of the NF2 protein.

Subject Categories:

  • Biochemistry
  • Medicine and Medical Research

Distribution Statement:

APPROVED FOR PUBLIC RELEASE