SLC26A5

This gene is a member of the SLC26A/SulP transporter family. It encodes a protein that is specifically expressed in outer hair cells (OHCs) of the cochlea and is essential in auditory processing. Intracellular anions are thought to act as extrinsic voltage sensors, which bind to this protein and trigger the conformational changes required for rapid length changes in OHCs. Mutations in this gene have been associated with non-syndromic hearing loss. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq]

solute carrier family 26, member 5 (prestin)

Motor protein that converts auditory stimuli to length changes in outer hair cells and mediates sound amplification in the mammalian hearing organ. Prestin is a bidirectional voltage-to-force converter, it can operate at microsecond rates. It uses cytoplasmic anions as extrinsic voltage sensors, probably chloride and bicarbonate. After binding to a site with millimolar affinity, these anions are translocated across the membrane in response to changes in the transmembrane voltage. They move towards the extracellular surface following hyperpolarization, and towards the cytoplasmic side in response to depolarization. As a consequence, this translocation triggers conformational changes in the protein that ultimately alter its surface area in the plane of the plasma membrane. The area decreases when the anion is near the cytoplasmic face of the membrane (short state), and increases when the ion has crossed the membrane to the outer surface (long state). So, it acts as an incomplete transporter. It swings anions across the membrane, but does not allow these anions to dissociate and escape to the extracellular space. Salicylate, an inhibitor of outer hair cell motility, acts as competitive antagonist at the prestin anion-binding site (By similarity).

Biological Process cochlea developmentIEA:Ensembl
Biological Process fructose transmembrane transportIEA:Ensembl
Biological Process negative regulation of ion transmembrane transportIEA:Ensembl
Biological Process positive regulation of cell motilityIEA:Ensembl
Biological Process positive regulation of cell sizeIEA:Ensembl
Biological Process regulation of cell shapeIEA:UniProtKB-KW
Biological Process regulation of membrane potentialIEA:Ensembl
Biological Process response to auditory stimulusIEA:Ensembl
Biological Process response to ischemiaIEA:Ensembl
Biological Process response to potassium ionIEA:Ensembl
Biological Process response to salicylic acidIEA:Ensembl
Biological Process response to saltIEA:Ensembl
Biological Process response to thyroid hormoneIEA:Ensembl
Biological Process response to xenobiotic stimulusIEA:Ensembl
Biological Process sensory perception of soundManual Assertion Based On ExperimentIMP:UniProtKB

Cell membrane
Lateral wall of outer hair cells.

Deafness, autosomal recessive, 61 (DFNB61):
A form of non-syndromic sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.