title: Detection of otosclerosis-specific measles virus receptor (cd46) protein isoforms.
abstract: Genetic predisposition of otosclerosis has long been suspected, but unclarified. Unique coexpression pattern of measles virus receptor (CD46) splicing isoforms in the human otic capsule is assumed, since otosclerosis is a measles virus-associated organ-specific disease. In order to identify CD46 involved in the pathogenesis of otosclerosis, we used representative groups of histologically diagnosed otosclerotic, nonotosclerotic, and normal stapes footplates (n = 109). Consecutive histopathological examinations and CD46-specific Western blot analysis were performed. Normal and nonotosclerotic stapes footplates showed consistent expression of the conventional c, d, e, f, and l CD46 isoforms. In contrast, four novel isoforms (os1-4) translated as intact proteins were additionally detected in each otosclerotic specimen. The study herein presented provides evidence for the otosclerosis-associated expression pattern of CD46. This finding might explain the organ-specific, virus-associated and autoimmune-inflammatory pathogenesis of otosclerosis. Regarding our current knowledge, this is the first report that confirms the presence of four new disease-specific protein variants of CD46.
authors.affiliation: Bajcsy-Zsilinszky Hospital, Department of Otolaryngology, Budapest, Hungary.
authors.forename: Balázs
authors.initials: B
authors.lastname: Liktor
authors.forename: Péter
authors.initials: P
authors.lastname: Csomor
authors.forename: Tamás
authors.initials: T
authors.lastname: Karosi