May 31, 2023

Researchers' findings provide possibility of reversing deafness in some

A pair of Wayne State University School of Medicine researchers have published findings that indicate the foundations for reversing profound deafness in certain types of patients.

Dennis Drescher, Ph.D., professor and director of Molecular Research, and Marian Drescher, Ph.D., associate professor, both in the Department of Otolaryngology - Head and Neck Surgery, recently published “Analysis of dysferlin direct interactions with putative repair proteins links apoptotic signaling to Ca2+ elevation via PDCD6 and FKBP8” in the International Journal of Molecular Sciences and “Protein interaction analysis by surface plasmon resonance” in Advanced Methods in Structural Biology.

The publications highlight proteins belonging to a group called ferlins. Two important representatives of this protein class are otoferlin, a protein necessary for hearing by triggering calcium needed for synaptic transmission in the inner-ear sensory cells, and dysferlin, a protein needed for the continuous repair of skeletal muscle tissue essential for ambulation and other muscular activities.

Otoferlin and dysferlin are remarkably similar in their amino acid sequence, prompting examination of molecular commonalities of biological function and dysfunction across organ systems. By employing the methods of confocal microscopy and surface plasmon resonance, or SPR, the Dreschers have related the cellular localization and strength of association of protein molecules essential to function in living cells.

Identification of the proteins that interact with otoferlin and dysferlin will permit pharmacological targeting of the binding proteins in patients, potentially reversing profound deafness of DFNB9 and of muscular dystrophy type 2B (LGMD2B).

The Dreschers have pioneered the study of neurotransmitters of hearing and balance as well as analogous biochemical systems in muscle for more than 40 years at Wayne State University with funding from the National Institutes of Health (Grants R01 DC000156 and R01 DC004076) and the Jain Foundation (Grant 25TTV) totaling more than $9 million.

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