Abstract: Provided are methods of transducing hair cells of the inner ear in a subject comprising administering to the subject an adeno-associated viral (AAV) vector comprising a nucleic acid sequence encoding a capsid comprising the amino acid sequence of SEQ ID NO: 1, wherein the AAV vector further comprises a heterologous nucleic acid sequence. Additionally, methods of treating, preventing, or inhibiting a cochlear disorder or balance disorder in a subject comprising administering the AAV vector to the subject are provided.

Abstract: The present technology relates generally to compositions and methods of preventing or treating diseases associated with mutant Cx26, Cx32, and/or Cx30 hemichannels. The present technology also relates to administering the anti-Cx26 hemichannel antibodies in effective amounts to treat a subject suffering from, or predisposed to, ectodermal dysplasia 2, Clouston type (OMIM No. 129500), Keratitis-ichthyosis-deafness syndrome (KIDS; OMIM No. 148210), and/or type X Charcot-Marie-Tooth neuropathy (CMTXl; OMIM No. 302800).

Abstract: The present invention provides a fully human antibody specifically inhibiting Connexin 26, characterized in that it is a recombinant immunoglobulin having the structure of scFv-Fc, wherein scFv refers to a single-chain antibody comprising a heavy chain variable region and a light chain variable region, the amino acid sequence of the heavy chain variable region is SEQ ID NO: 1, the amino acid sequence of the light chain variable region is SEQ ID NO: 2, and Fc refers to constant region. The present invention uses the first 41-56 amino acid sequence of extracellular region of Connexin26 as an antigen, the sequence thereof is KEVWGDEQADFVCNTL. Through the biochemical analysis and immunofluorescence identification of the antibody obtained by single-chain antibody phage display library and screening technology, it was confirmed that the antibody provided by the present invention could specifically recognize Connexin 26 and inhibit its hemichannel activity.