Key protein of hearing discovered
Marburg research team identifies molecular connecting element of the outer hair cells in the inner ear
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A research team at Philipps University Marburg has identified a previously unknown protein that plays a central role in human hearing. According to the study, the protein TMEM145 is an essential component of the outer hair cells in the inner ear - the sensory cells that amplify sound and thus enable precise hearing of very soft sounds. The team led by first author Dennis Derstroff and Prof. Dr. Katrin Reimann from the Department of Otorhinolaryngology and Prof. Dr. Dominik Oliver from the Institute of Physiology and pathophysiology was able to show that TMEM145 forms a ring-shaped structure at the tips of the sensory hairs (stereocilia), where it organizes the mechanical connection to the so-called tectorial membrane. This fine circular structure lies in the inner ear above the hair cells and transmits the sound vibrations to their sensory hairs. If TMEM145 is missing, the hair cells lose this mechanical connection: In genetically modified mice, this leads to severe hearing loss and loss of the amplification function of the cochlea
Biological amplifiers in the inner ear
The results provide new insights into a previously only partially understood mechanism of hearing. In the inner ear, sound first causes a mechanical vibration, which is then translated into electrical signals, the language of the brain. The outer hair cells act as biological amplifiers of the mechanical sound vibration. The prerequisite for this is a stable mechanical connection between their stereocilia and the tectorial membrane. "Our results show that TMEM145 functions as a molecular anchoring and organization center that enables the mechanical excitation of the hair cells - a prerequisite for the amplifier function of the inner ear to work at all," says study leader Prof. Dr. Katrin Reimann.
Therapeutic time window
In the long term, the results could also be relevant for the diagnosis and treatment of hearing disorders. Several proteins that work together with TMEM145 are already known to cause genetic hearing disorders. TMEM145 could therefore be another candidate gene in the genetic diagnosis of hearing loss in the future. At the same time, the mouse model suggests that there could be a therapeutic window of opportunity before permanent damage occurs - for example, for gene therapy approaches such as those already being tested for other genetically caused forms of sensorineural hearing loss. The study shows how basic molecular research in Marburg is leading to new perspectives for the understanding and treatment of hearing disorders.
Interlinked medical research
The work is an example of the close integration of clinical medicine and basic molecular research at the University of Marburg. Researchers from the Department of Otorhinolaryngology and the Institute of Physiology and Pathophysiology are working closely together to understand the biological basis of hearing and investigate its significance for hearing disorders. This collaboration is complemented by an international network of partners, including researchers from Harvard Medical School in Boston and University College London (UCL). "This international cooperation combines different scientific perspectives - from clinical experience to molecular cell biology and genetic models - and makes it possible to comprehensively investigate complex mechanisms of hearing," says Prof. Reimann. The combination of interdisciplinary research in Marburg and close collaboration with leading international centers was a decisive factor in the success of the project. The study was funded by the German Research Foundation (DFG), the British Medical Research Council and the National Institutes of Health (USA).
Note: This article has been translated using a computer system without human intervention. LUMITOS offers these automatic translations to present a wider range of current news. Since this article has been translated with automatic translation, it is possible that it contains errors in vocabulary, syntax or grammar. The original article in German can be found here.
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Genetic diseases once considered untreatable are now at the center of innovative therapeutic approaches. Research and development of gene therapies in biotech and pharma aim to directly correct or replace defective or missing genes to combat disease at the molecular level. This revolutionary approach promises not only to treat symptoms, but to eliminate the cause of the disease itself.
Topic world Gene therapy
Genetic diseases once considered untreatable are now at the center of innovative therapeutic approaches. Research and development of gene therapies in biotech and pharma aim to directly correct or replace defective or missing genes to combat disease at the molecular level. This revolutionary approach promises not only to treat symptoms, but to eliminate the cause of the disease itself.
