Genetic deafness is one of the most prevalent inherited sensory disorders, affecting about 1 in 2,000 children. Mutations in the connexin 26 gene have been associated with autosomal recessive non-syndromic deafness (DFNB1)¹. The connexin 26 gene is a member of the connexin family of genes, which encode intercellular channels comprising gap junctions², and it is abundantly expressed in the organ of Corti¹,³. Here we test the channel-forming ability of mutant connexin 26 proteins using a well-characterized in vitro system for functional expression of connexin channels⁴. We find that mutant connexin 26 proteins can act as dominant inhibitors of wild-type connexin 26 channel activity.

1. Department of Cell Biology, Harvard Medical School, Boston, Massachusetts 02115, USA
2. Centre for Cutaneous Research, St Bartholomews and the Royal London Hospital, London E1 2AT, UK
3. Department of Neurobiology, Harvard Medical School, Boston, Massachusetts 02115, USA
   e-mail: Email: dpaul@hms.med.harvard.edu