TY - JOUR
T1 - Hearing impairment
T2 - A panoply of genes and functions
AU - Dror, Amiel A.
AU - Avraham, Karen B.
N1 - Funding Information:
Research on hereditary hearing loss in the Karen B. Avraham laboratory is supported by NIH grant R01DC005641 from the National Institute of Deafness and Communication Disorders, Israel Science Foundation (1486/07), the Israel Ministry of Health (MOH), and the European Commission FP6 Integrated Project Eumodic 037188.
PY - 2010/10/21
Y1 - 2010/10/21
N2 - Research in the genetics of hearing and deafness has evolved rapidly over the past years, providing the molecular foundation for different aspects of the mechanism of hearing. Considered to be the most common sensory disorder, hearing impairment is genetically heterogeneous. The multitude of genes affected encode proteins associated with many different functions, encompassing overarching areas of research. These include, but are not limited to, developmental biology, cell biology, physiology, and neurobiology. In this review, we discuss the broad categories of genes involved in hearing and deafness. Particular attention is paid to a subgroup of genes associated with inner ear gene regulation, fluid homeostasis, junctional complex and tight junctions, synaptic transmission, and auditory pathways. Overall, studies in genetics have provided research scientists and clinicians with insight regarding practical implications for the hearing impaired, while heralding hope for future development of therapeutics.
AB - Research in the genetics of hearing and deafness has evolved rapidly over the past years, providing the molecular foundation for different aspects of the mechanism of hearing. Considered to be the most common sensory disorder, hearing impairment is genetically heterogeneous. The multitude of genes affected encode proteins associated with many different functions, encompassing overarching areas of research. These include, but are not limited to, developmental biology, cell biology, physiology, and neurobiology. In this review, we discuss the broad categories of genes involved in hearing and deafness. Particular attention is paid to a subgroup of genes associated with inner ear gene regulation, fluid homeostasis, junctional complex and tight junctions, synaptic transmission, and auditory pathways. Overall, studies in genetics have provided research scientists and clinicians with insight regarding practical implications for the hearing impaired, while heralding hope for future development of therapeutics.
UR - http://www.scopus.com/inward/record.url?scp=77957911916&partnerID=8YFLogxK
U2 - 10.1016/j.neuron.2010.10.011
DO - 10.1016/j.neuron.2010.10.011
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C2 - 20955936
AN - SCOPUS:77957911916
SN - 0896-6273
VL - 68
SP - 293
EP - 308
JO - Neuron
JF - Neuron
IS - 2
ER -