Anselm A . Zdebik , Philine Wangemann and Thomas J . Jentsch Genetic Disease and Mouse Models Potassium

Philine Wangemann and Thomas J . Jentsch

Potassium ion movement in the inner ear: insights from genetic disease and mouse models.

Sensory transduction in the cochlea and vestibular labyrinth depends on fluid movements that deflect the hair bundles of mechanosensitive hair cells. Mechanosensitive transducer channels at the tip of the hair cell stereocilia allow K(+) to flow into cells. This unusual process relies on ionic gradients unique to the inner ear. Linking genes to deafness in humans and mice has been instrumental ...

KCNJ 10 ( Kir 4 . 1 ) potassium channel knockout abolishes endocochlear potential DANIEL

Marcus, Daniel C., Tao Wu, Philine Wangemann, and Paulo Kofuji. KCNJ10 (Kir4.1) potassium channel knockout abolishes the endocochlear potential. Am J Physiol Cell Physiol 282: C403–C407, 2002; 10.1152/ajpcell.00312.2001.— Stria vascularis of the cochlea generates the endocochlear potential and secretes K . K is the main charge carrier and the endocochlear potential the main driving force for th...

Molecular structure and physiological function of chloride channels.

Cl- channels reside both in the plasma membrane and in intracellular organelles. Their functions range from ion homeostasis to cell volume regulation, transepithelial transport, and regulation of electrical excitability. Their physiological roles are impressively illustrated by various inherited diseases and knock-out mouse models. Thus the loss of distinct Cl- channels leads to an impairment o...

Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV.

Human Bartter syndrome IV is an autosomal recessive disorder characterized by congenital deafness and severe renal salt and fluid loss. It is caused by mutations in BSND, which encodes barttin, a beta-subunit of ClC-Ka and ClC-Kb chloride channels. Inner-ear-specific disruption of Bsnd in mice now reveals that the positive potential, but not the high potassium concentration, of the scala media ...