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Article Synopsis
  • Myh1 is identified as a mouse gene linked to deafness, with its role in the auditory system still unclear; knockout mice show significant hearing impairment and abnormal hair cell function.
  • Research shows that MYH1 variants in humans contribute to non-progressive hearing loss, with some individuals also experiencing osteopenia.
  • Structural and functional analysis indicates that MYH1 variants disrupt regular activity in outer hair cells, highlighting the gene's essential role in hearing and its genetic connection to hearing loss in affected families.
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Verification of Outer Hair Cell Motor Protein, Prestin, as a Serological Biomarker for Mouse Cochlear Damage.

Int J Mol Sci

July 2024

Department of Otolaryngology-Head and Neck Surgery, Feinberg School of Medicine, Chicago Campus, Northwestern University, Chicago, IL 60611, USA.

The motor protein prestin, found in the inner ear's outer hair cells (OHCs), is responsible for high sensitivity and sharp frequency selectivity in mammalian hearing. Some studies have suggested that prestin could be a serological biomarker for cochlear damage, as OHCs are highly vulnerable to damage from various sources. However, the reported data are inconsistent and lack appropriate negative controls.

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Prestin in Human Perilymph, Cerebrospinal Fluid, and Blood as a Biomarker for Hearing Loss.

Otolaryngol Head Neck Surg

December 2024

Christian Doppler Laboratory for Inner Ear Research, Department of Otorhinolaryngology-Head and Neck Surgery, Vienna General Hospital, Medical University of Vienna, Vienna, Austria.

Objective: Determining the concentration of prestin in human blood, cerebrospinal fluid (CSF), and perilymph (PL), and evaluating its suitability as a clinical biomarker for sensori-neural hearing loss (SNHL).

Study Design: Human blood, CSF, and PL samples were intraoperatively collected from 42 patients with tumors of the internal auditory canal or with intracochlear tumors undergoing translabyrinthine or middle fossa tumor removal. Prestin concentration was measured using enzyme-linked immunosorbent assay and linear regression analyses were performed to investigate its associations with audiological as well as vestibular test results.

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Objective: Prestin is an outer hair cell (OHC) protein responsible for increasing cochlear sensitivity and has been proposed as a biomarker. We aimed to evaluate whether the serum prestin level is related to the severity of chronic sensorineural hearing loss (SNHL).

Methods: Ninety subjects were recruited from the patient base at Samarra public hospitals and clinics in Iraq from January to October of 2022.

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Elevator-like movements of prestin mediate outer hair cell electromotility.

Nat Commun

November 2023

Department of Neurophysiology, Institute of Physiology and Pathophysiology, Philipps University Marburg, 35037, Marburg, Germany.

The outstanding acuity of the mammalian ear relies on cochlear amplification, an active mechanism based on the electromotility (eM) of outer hair cells. eM is a piezoelectric mechanism generated by little-understood, voltage-induced conformational changes of the anion transporter homolog prestin (SLC26A5). We used a combination of molecular dynamics (MD) simulations and biophysical approaches to identify the structural dynamics of prestin that mediate eM.

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