Stereocilin (STRC)

Stereocilin is a structural protein encoded by the STRC gene and is found localized to the hair bundles of sensory hair cells in the inner ear[4][2][3]. Its main function is to link the tips of neighboring stereocilia, which ensures correct mechanical coupling of these structures to the tectorial membrane, crucial for sensation and amplification of sound[2][1][3]. Stereocilin is essential for certain auditory functions such as detecting sound intensity and frequency; loss-of-function mutations in STRC disrupt the cohesion and mechanical properties of hair bundles, causing autosomal recessive nonsyndromic hearing loss, especially DFNB16[2][3][4][5]. Large deletions may also include the CATSPER2 gene, leading to a syndromic form of hearing loss and male infertility[4][5]. Stereocilin is not a conventional drug target (such as receptor, enzyme, transporter), but has recently been investigated for gene therapy approaches aimed at correcting hereditary hearing loss due to STRC mutations[6]. Detection and diagnosis are complicated by the presence of a highly similar STRC pseudogene, requiring advanced genetic methods[3]. There are no known interacting drugs or pharmacological mechanisms of action as of now.

Other names

STRC proteinSTRC gene productStereocilin protein