News

Corey paper on the dimeric structure and physiology of the TMC1 channel was selected for Neuron's Best of 2017-2018.

February 6, 2019
David Corey and Jeffrey Holt paper, TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells, was selected for Neuron’s Best of 2017-2018. The choices were made based on the most accessed papers in the year from Oct 2017 to Sept 2018. The paper was also included in... Read more about Corey paper on the dimeric structure and physiology of the TMC1 channel was selected for Neuron's Best of 2017-2018.

New published paper with support from the Bertarelli Program: Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders

January 24, 2019

Authors: Carl A. Nist-Lund, Bifeng Pan, Amy Patterson, Yukako Asai, Tianwen Chen, Wu Zhou, Hong Zhu, Sandra Romero, Jennifer Resnik, Daniel B. Polley, Gwenaelle S. Géléoc, & Jeffrey R. Holt

Abstract: Fifty percent of inner ear disorders are caused by genetic mutations. To develop treatments for genetic inner ear disorders, we designed gene replacement therapies using synthetic adeno-associated viral vectors to deliver the coding sequence for Transmembrane Channel-Like (Tmc) 1 or 2 into sensory hair cells of mice with hearing and balance deficits due to mutations in ... Read more about New published paper with support from the Bertarelli Program: Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders

Scientific American Article Out of the Silence: Gene Therapy Tackles a Common Birth Defect: Deafness by Dina Fine Maron

December 4, 2018

Hearing loss is one of the most common birth defects, and genetic flaws are frequently the cause. After false starts, researchers are making progress toward treating deafness with gene therapy. This in-depth piece explores the latest research in gene therapy as a way to restore hearing and profiles the work of David Corey (HMS), Bence György (HMS), Jeffrey Holt (HMS/Boston Children’s) and Gwenaëlle Geleoc (HMS/Boston Children’s). Article features current and former Bertarelli-funded PIs, including David Corey and Jeffrey Holt....

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Featured article in Neuron: TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells

August 23, 2018
Preview by Peter G. Barr-Gillespie

Honing In on TMC as the Hair Cell's Transduction Channel

The identity of the inner ear's transduction channel has bedeviled auditory neuroscientists for decades. In this issue of Neuron, Pan et. al. (2018) report the most convincing evidence to date implicating the transmembrane channel-like (TMC) proteins as forming the pore of the transduction channel.

Authors: Bifeng Pan...

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Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1

August 14, 2018
Recent work has demonstrated that transmembrane channel-like 1 protein (TMC1) is an essential component of the sensory transduction complex in hair cells of the inner ear. A closely related homolog, TMC2, is expressed transiently in the neonatal mouse cochlea and can enable sensory transduction in Tmc1-null mice during the first postnatal week. Both TMC1 and TMC2 are expressed at adult stages in mouse vestibular hair cells. The extent to which TMC1 and TMC2 can substitute for each other is unknown. Several biophysical differences between TMC1 and TMC2 suggest these proteins perform... Read more about Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1

Call for Proposals: Bertarelli Collaborative Awards for Sensory Disorders

August 13, 2018

Submit by September 1, 2018, 5:00 p.m. EST

The Bertarelli Program in Translational Neuroscience and Neuroengineering is now accepting applications for Bertarelli Collaborative Awards for Sensory Disorders. The program, funded by the Bertarelli Foundation, will bridge the gap between basic and translational neuroscience through research oriented towards translational opportunities and through creation of stronger ties among scientists, engineers and...

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Article featuring Taralyn Tan, Bertarelli-funded Curriculum Fellow

August 9, 2018

On a wintry Thursday morning, Taralyn Tan, PhD ’16, woke before the crack of dawn to prepare for a packed day...she headed over to the Warren Alpert Building on the HMS campus, where she sat in on the Discipline of Neuroscience class, a new foundational class in the School’s graduate Program in Neuroscience (PiN). For three hours, she watched and listened, gauging how the curriculum changes she’d helped implement were working with students and faculty....

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Sensory Neuron Diversity in the Inner Ear Is Shaped by Activity

August 2, 2018
In the auditory system, type I spiral ganglion neurons (SGNs) convey complex acoustic information from inner hair cells (IHCs) to the brainstem. Although SGNs exhibit variation in physiological and anatomical properties, it is unclear which features are endogenous and which reflect input from synaptic partners. Using single-cell RNA sequencing, we derived a molecular classification of mouse type I SGNs comprising three subtypes that express unique combinations of Ca2+ binding proteins, ion channel...
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