Molecular-Functional Comparison of the Dysferlin Membrane Repair Complex with the Otoferlin Synaptic Complex

Dennis G. Drescher, PhD

Wayne State University

Dr. Drescher is Professor and Director of Molecular Biology in the Department of Otolaryngology and Biochemistry-Molecular Biology at Wayne State University (Detroit, MI).

Research Projects

Objective: 
To compare the molecular function of dysferlin and otoferlin.

Genetically-defective dysferlin causes LGMD2B muscular dystrophy and mutated otoferlin causes DFNB9 deafness. Both proteins normally function by forming complexes with other proteins that either repair muscle (dysferlin) or promote hearing through sound-signal transmission (otoferlin). We are investigating the molecular role of dysferlin in muscle, guided by known ferlin interactions in inner ear hair cells, in an examination of the generalized molecular properties of dysferlin across tissues. By using biochemical and molecular-biological binding methods, we are detecting the strongest protein-protein interactions and thus identifying key protein players likely to be part of the dysferlin (and otoferlin) protein complexes that are responsible for membrane repair and fusion. Understanding these protein complexes through a comparative approach further guides therapeutic intervention.