SCHWANNOMIN - NEW INSIGHTS INTO THIS MEMBER OF THE BAND-4.1 SUPERFAMILY

Neurofibromatosis type 2 (NF2) is an autosomal dominant disease charac terized by the development of central nervous system tumours. The NM g ene was recently cloned and found to encode a protein, schwannomin (or merlin), with homology to the band 4.1 superfamily. This superfamily of proteins includes ezrin, moesin, radixin, and talin, as well as sev eral protein tyrosine phosphatases. How does a cytoskeleton-associated protein act as a tumour suppressor? While this fundamental question r emains unanswered, recent studies have begun to address key questions regarding the function of schwannomin. In this review, we examine what is known about the band 4.1 superfamily and how this information pert ains to schwannomin. In addition, we summarize recent studies of schwa nnomin itself.