Inside the Holt/Géléoc Lab

Gene therapy strategies lend hope to hearing loss treatment
Story by Suzanne Day. Photo by Garyfallia Pagonis.
From the Fall 2014 issue of
Harvard Otolaryngology magazine. For inquiries or to be added to our mailing list, please contact
When Jeffrey Holt, Ph.D., and his wife Gwenaelle Géléoc, Ph.D., were offered faculty positions in the Harvard Medical School Department of Otolaryngology four years ago, they made the difficult decision to move their research efforts—and their lives together—from Charlottesville, Va., to Boston.
At the time, they were excited about a possible hearing research breakthrough, in which they believed they had found two genes and proteins responsible for hair cell transduction in the inner ear. The two scientists, who met as postdocs at Mass General, had served ten years on the faculty at the University of Virginia, where they had grown a highly productive research program in inner ear physiology.
“We really struggled with the decision to move, because we loved Charlottesville and weren’t looking for a change,” Dr. Holt said. “But we recognized that this was an important discovery, and we were excited about the opportunity for collaboration in Boston, a city where the science is just phenomenal.”
Drs. Holt and Géléoc have since made substantial contributions in the area of gene therapy for hearing loss. In their lab at Boston Children’s Hospital, they have identified TMC1 and TMC2 as components of the hair cell transduction channel, key molecules required for auditory processing, and have designed a gene therapy trial using viral vectors to correct mutations in TMC1. They are also developing similar gene therapy strategies for Usher syndrome, a genetic condition that affects hearing and vision.
“We’re developing vectors to target the hair cells of the inner ear and also the photoreceptors of the retina, which are also affected by Usher syndrome,” Dr. Géléoc said. “We’re working to find ways to counteract the progression of deafness and retinal degeneration.”
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