Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle.

R Klocke, K Steinmeyer, TJ Jentsch… - Journal of Biological …, 1994 - ASBMB
The muscular chloride channel (ClC-1) is essential for a normal excitability of mature
mammalian muscle fibers; inactivation of the corresponding gene by mutations leads to
hyperexcitability of muscle, the hallmark of the disease myotonia. In the mouse, there is very
little ClC-1 mRNA in myotubes, and its concentration increases steeply during postnatal
development, suggesting a role of the motor nerve in ClC-1 expression. We investigated the
response of the expression of the corresponding gene Clc-1 to different patterns of muscle …