T. Kharkovets et al., KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway, P NAS US, 97(8), 2000, pp. 4333-4338
Citations number
45
Categorie Soggetti
Multidisciplinary
Journal title
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Mutations in the potassium channel gene KCNQ4 underlie DFNA2, an autosomal
dominant form of progressive hearing loss in humans. In the mouse cochlea,
the transcript has been found exclusively in the outer hair cells. By using
specific antibodies, we now show that KCNQ4 is situated at the basal membr
ane of these sensory cells. In the vestibular organs, KCNQ4 is restricted t
o the type I hair cells and the afferent calyx-like nerve endings ensheathi
ng these sensory cells. Several lines of evidence suggest that KCNQ4 underl
ies the I-K,I-n, and g(K,L) currents that have been described in the outer
and type I hair cells, respectively, and that are already open at resting p
otentials. KCNQ4 is also expressed in neurons of many, but not all, nuclei
of the central auditory pathway, and is absent from most other brain region
s. It is present, e.g., in the cochlear nuclei, the nuclei of the lateral l
emniscus, and the inferior colliculus, This is the first ion channel shown
to be specifically expressed in a sensory pathway. Moreover, the expression
pattern of KCNQ4 in the mouse auditory system raises the possibility of a
central component in the DFNA2 hearing loss.