Usher Protein 1 Antibody from MyBioSource.com

Usher syndrome, an autosomal recessive heterogeneous disorder, is the most common cause of deafness and blindness in adults and affect 3-6% children born with hearing impairment. The affected individuals have sensory-neural deficiencies at birth and subsequently develop progressive retinitis pigmentosa (RP). Three forms of Usher syndrome have been characterized, Usher Type 1, II and III which can be distinguish based on severity of hearing loss and vestibular involvement. Type I patents are profoundly deaf while type II (most common from of Usher syndrome) patients are mildly deaf but have normal in vestibular responses (1). Ush2A gene has been mapped on a 1 mega base pair interval between marker D1S474 and AFM144XF2. The Usher 2A gene expresses an extra cellular matrix protein that resembles unconventional Myosin and do not appear to have any un-conventional functional correlations. The human Usher Syndrome 2A (Ush2A) protein has 1272 amino acid (Approximate MW 153 kDa) with several functional domains (Laminin type EGF like domain; Laminin-Type epidermal growth factor like domain and fibronectin type 3 like domain). The protein has a single transmembrane domain that anchor the protein to the cell membrane, the rest of the protein stays outside the cell. The Ush2A protein is approximately 153 kDa (1272 amino acids) with multiple conserved domains that is mainly expressed in RPEs. The Anti-Ush2A-selective antibodies were generated against three regions (Ush-101AP epitope: 155-172; Ush-112AP epitope: 189-204 and Ush-121AP epitope: Cterminal peptide). These epitope are unique to Ush2A protein. The polyclonal antibody strongly labels a 186 kDa protein in RPE cell extracts. Anti-Ush2A-selective antibodies are also available in affinity-purified form for confocal, Western blotting and immunocytochemical analyses