Protein Discovery in the Development of New Hearing Hair Cells Could Lead to Treatments for Hearing Loss

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Hearing Hair Cells

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A surface area view of the organ of hearing (cochlea) from a mouse, utilizing confocal microscopy. The sensory cells are called hair cells due to the fact that of their apical forecasts (stereocilia) which move from stimulation by noise. Credit: University of Maryland School of Medicine

Researchers at the University of Maryland School of Medicine (UMSOM) have actually performed a research study that has actually figured out the function that a vital protein plays in the advancement of hair cells. These hair cells are crucial for hearing. Some of these cells magnify sounds that entered into the ear, and others change acoustic waves into electrical signals that take a trip to the brain. Ronna Hertzano, MD, PhD, Associate Professor in the Department of Otorhinolaryngology Head and Neck Surgery at UMSOM and Maggie Matern, PhD, a postdoctoral fellow at Stanford University, showed that the protein, called GFI1, might be crucial for identifying whether an embryonic hair cell develops into a practical adult hair cell or ends up being a various cell that operates more like an afferent neuron or nerve cell.

The research study was released in the journal Development, and was performed by physician-scientists and scientists at the UMSOM Department of Otorhinolaryngology Head and Neck Surgery and the UMSOM Institute for Genome Sciences (IGS), in cooperation with scientists at the Sackler School of Medicine at Tel Aviv University in Israel.

Hearing depends on the correct performance of specialized cells within the inner ear called hair cells. When the hair cells do not establish effectively or are harmed by ecological tensions like loud sound, it leads to a loss of hearing function.

In the United States, the occurrence of hearing loss doubles with every 10-year boost in age, impacting about half of all grownups in their 70s and about 80 percent of those who are over age 85. Researchers have actually been concentrating on explaining the developmental actions that cause a practical hair cell, in order to possibly produce brand-new hair cells when old ones are harmed.

To perform her newest research study, Dr. Hertzano and her group made use of innovative techniques to study gene expression in the hair cells of genetically customized newborn mice that did not produce GFI1. They showed that, in the lack of this crucial protein, embryonic hair cells stopped working to advance in their advancement to end up being completely practical adult cells. In truth, the genes revealed by these cells suggested that they were most likely to become neuron-like cells.

“Our findings explain why GFI1 is critical to enable embryonic cells to progress into functioning adult hair cells,” stated Dr. Hertzano. “These data also explain the importance of GFI1 in experimental protocols to regenerate hair cells from stem cells. These regenerative methods have the potential of being used for patients who have experienced hearing loss due to age or environmental factors like exposure to loud noise.”

Dr. Hertzano very first ended up being thinking about GFI1 while finishing her M.D., Ph.D. at Tel Aviv University. As part of her argumentation, she found that the hearing loss arising from anomalies in another protein called POU4F3 appeared to mainly arise from a loss of GFI1 in the hair cells. Since then, she has actually been performing research studies to find the function of GFI1 and other proteins in hearing.  Other research study groups in the field are now checking these proteins to identify whether they can be utilized as a “cocktail” to restore lost hair cells and bring back hearing.

“Hearing research has been going through a Renaissance period, not only from advances in genomics and methodology, but also thanks to its uniquely collaborative nature among researchers,” stated Dr. Herzano.

The brand-new research study was moneyed by the National Institute on Deafness and Other Communication Disorders (NIDCD) which belongs to the National Institutes of Health (NIH). It was likewise moneyed by the Binational Scientific Foundation (BSF).

“This is an exciting new finding that underscores the importance of basic research to lay the foundation for future clinical innovations,” stated E. Albert Reece, MD, PhD, MBA, Executive Vice President for Medical Affairs, UM Baltimore, and the John Z. and Akiko K. Bowers Distinguished Professor and Dean, University of Maryland School of Medicine. “Identifying the complex pathways that lead to normal hearing could prove to be the key for reversing hearing loss in millions of Americans.”

Reference: “GFI1 functions to repress neuronal gene expression in the developing inner ear hair cells” by Maggie S. Matern, Beatrice Milon, Erika L. Lipford, Mark McMurray, Yoko Ogawa, Andrew Tkaczuk, Yang Song, Ran Elkon and Ronna Hertzano, 11 September 2020, Development.
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