Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders
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چکیده
منابع مشابه
Retracted: Experimental Study of Local Inner Ear Gene Therapy for Controlling Autoimmune Sensorineural Hearing Loss
[This retracts the article DOI: 10.1155/2014/134658.].
متن کاملThe transmembrane inner ear (Tmie) protein is essential for normal hearing and balance in the zebrafish.
Little is known about the proteins that mediate mechanoelectrical transduction, the process by which acoustic and accelerational stimuli are transformed by hair cells of the inner ear into electrical signals. In our search for molecules involved in mechanotransduction, we discovered a line of deaf and uncoordinated zebrafish with defective hair-cell function. The hair cells of mutant larvae fai...
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In order to identify the inner ear autoantibodies, sera of 195 patients with sensorineural hearing loss and/or vertigo were reached with bovine inner ear antigen by western blotting method. Positive reaction was seen at several molecular weights, in which 33-35 kD, 42 kD and 68 kD were highly reactive. In moderate to severe hearing loss patients, positive reactions with 33-35 kD, 42 kD, 68 kD a...
متن کاملCell- and gene-therapy approaches to inner ear repair.
Sensorineural hearing loss is the most common sensory disorder in humans. It is primarily due to the degeneration of highly specialised mechanosensory cells in the cochlea, the so-called hair cells. Hearing problems can also be caused or further aggravated by the death of auditory sensory neurons that convey the information from the hair cells to the brain stem. Despite the discovery of stem/pr...
متن کاملHave You Heard? Viral-Mediated Gene Therapy Restores Hearing
Genetic loss of VGLUT3 in cochlear inner hair cells results in profound deafness. In this issue of Neuron, Akil et al. (2012) show that AAV-mediated introduction of wild-type VGLUT3 in the genetically deaf mouse cochlea results in significantly improved hearing.
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ژورنال
عنوان ژورنال: Nature Communications
سال: 2019
ISSN: 2041-1723
DOI: 10.1038/s41467-018-08264-w