Article
Cockayne syndrome group B (Csb) and group a (Csa) deficiencies predispose to hearing loss and cochlear hair cell degeneration in mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 11 Mar 2015
Nagtegaal A Paul, Rainey Robert N, van der Pluijm Ingrid, Brandt Renata M C, van der Horst Gijsbertus T J, Borst J Gerard G, Segil Neil
Abstract excerpt
Sensory hair cells in the cochlea, like most neuronal populations that are postmitotic, terminally differentiated, and non-regenerating, depend on robust mechanisms of self-renewal for lifelong survival. We report that hair cell homeostasis requires a specific sub-branch of the DNA damage nucleotide excision repair pathway, termed transcription-coupled repair (TCR). Cockayne syndrome (CS), caused by defects in...
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