The Beethoven mouse, like its namesake the classical music composer Ludwig von Beethoven, suffers progressive hearing loss and eventual profound deafness. In this issue, Pan et al. examined sensory transduction in inner ear hair cells of Beethoven mice, which carry a point mutation in Transmembrane channel-like 1 gene (Tmc1). They report reduced calcium permeability and reduced single-channel conductance in Beethoven hair cells relative to hair cells that expressed wild type Tmc1. The Beethoven data demonstrate that TMC1 is a component of the hair cell transduction channel. The authors found that a closely related homolog, TMC2, also functions as a component of the transduction channel. The image shows Ludwig von Beethoven as portrayed by Joseph Stieler (1820). A cross-section of the ear, including external ear, middle, and inner ear, appears below. Within the inner ear is the spiral-shaped cochlea. The inset below shows the sensory organ, or organ of Corti. At the bottom right is a scanning electron micrograph of a hair bundle from an inner hair cell, which was the main focus of the Pan et al. study. Cover montage by Emily Mills.
Tuesday, August 27, 2013
The Beethoven mouse - a key to auditory transduction
I have to pass on this great montage cover and its legend from the journal Neuron. The article referenced described a gene mutation that leads to progressive hearing loss.