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Publication : NeuroD-null mice are deaf due to a severe loss of the inner ear sensory neurons during development.

First Author  Kim WY Year  2001
Journal  Development Volume  128
Issue  3 Pages  417-26
PubMed ID  11152640 Mgi Jnum  J:66787
Mgi Id  MGI:1929186 Doi  10.1242/dev.128.3.417
Citation  Kim WY, et al. (2001) NeuroD-null mice are deaf due to a severe loss of the inner ear sensory neurons during development. Development 128(3):417-26
abstractText  A key factor in the genetically programmed development of the nervous system is the death of massive numbers of neurons. Therefore, genetic mechanisms governing cell survival are of fundamental importance to developmental neuroscience. We report that inner ear sensory neurons are dependent on a basic helix-loop-helix transcription factor called NeuroD for survival during differentiation. Mice lacking NeuroD protein exhibit no auditory evoked potentials, reflecting a profound deafness. DiI fiber staining, immunostaining and cell death assays reveal that the deafness is due to the failure of inner ear sensory neuron survival during development. The affected inner ear sensory neurons fail to express neurotrophin receptors, TrkB and TrkC, suggesting that the ability of NeuroD to support neuronal survival may be directly mediated through regulation of responsiveness to the neurotrophins.
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