Glutaredoxin domain-containing cysteine-rich protein 1 (GRXCR1)

GRXCR1 (Glutaredoxin domain-containing cysteine-rich protein 1) is a protein specifically expressed in cochlear hair cells and is essential for the normal formation of stereocilia, the actin-based mechanosensory structures required for detecting sound in the inner ear. Mutations in GRXCR1 cause nonsyndromic recessive hearing loss, designated DFNB25. While the central region of GRXCR1 is similar to glutaredoxin enzymes, which usually catalyze reversible glutathionylation of proteins, mammalian GRXCR1 lacks classical glutaredoxin enzymatic activity but may mediate critical protein–protein interactions within the stereocilia, influencing actin organization. Its main biological role is in auditory perception, where loss of function leads to severe hearing deficits due to defective stereocilia morphogenesis. There are no approved drugs or established pharmacological interventions targeting GRXCR1, but genetic testing for mutations is used as a biomarker in hereditary hearing loss cases.

Other names

DFNB25Glutaredoxin, cysteine rich 1Glutaredoxin domain-containing cysteine-rich protein 1GRXCR1

None established. There are currently no drugs targeting GRXCR1