Pejvakin (PJVK)

DFNB59 consists of 7 exons spanning 9.8 kb of genomic sequence. Exon organization between the pejvakin and DFNA5 genes throughout the region of similarity is identical, indicating that these 2 genes share a common origin.The 352-amino acid pejvakin protein shares significant similarity with the DFNA5 protein in the respective N-terminal regions, with 32% identity over a stretch of 250 amino acids.The pejvakin sequence contains a nuclear localization signal (residues 249-258) and a zinc-binding motif (residues 305-331). Pejvakin belongs to a family of proteins, including DFNA5 and the gasdermins, found only in vertebrates. Immunohistofluorescence microscopy detected pejvakin in the cell bodies of neurons of the afferent auditory pathway.