A NOVEL TYPE OF MYOSIN ENCODED BY THE MOUSE DEAFNESS GENE SHAKER-2

The mouse recessive deafness mutation, shaker-2(sh-2), represents a pl ausible model for an autosomal recessive form of human non-syndromic g enetic deafness, DFNB3. Here we report the use of a positional cloning approach to show that the gene mutated in sh-2 mice encodes a novel t ype of unconventional myosin, A G-to-A transition changing cysteine to tyrosine in the conserved actin binding domain is detected in sh-2 bu t absent in laboratory strains and wild mice belonging to different mo use subspecies and species. This suggests that the novel myosin gene i s a strong candidate for DFNB3. (C) 1998 Academic Press.