ClCl-- channel and channel and ClCl-- CHANNELOPATHIES CHANNELOPATHIES

•Properties of Cl- channels

•Cl- dependent myotonia

Skeletal muscle ClSkeletal muscle Cl-- channel channel

Skeletal muscle ClSkeletal muscle Cl-- channel channel

Skeletal muscle ClSkeletal muscle Cl-- channel channel

Cl- channel blocker,9-anthracene carboxylic acid (9-AC)

ClCl-- channel selectivity channel selectivity

ClCl-- channel activation channel activation

ClCl-- channel deactivation channel deactivation

Single ClSingle Cl-- channel conductance channel conductance

ClCl-- channel is a dimer with two pores channel is a dimer with two pores

Nucleotide dependency of ClNucleotide dependency of Cl-- channel channel

ATP

AMP

CONGTPIMPcAMP

Voltage-sensitive ClVoltage-sensitive Cl-- channel family channel family

Molecular structure of Voltage-sensitive ClMolecular structure of Voltage-sensitive Cl-- channelchannel

ClCl-- channel Family channel Family

Physiological role of the CFTR channel - Physiological role of the CFTR channel - PancreasPancreas

Physiological role of the CFTR channel – Physiological role of the CFTR channel – Sweat ductSweat duct

The CFTR channelThe CFTR channel

The CFTR channelThe CFTR channel

The CFTR channelThe CFTR channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonia from ClMyotonia from Cl-- channel channel

Myotonic runMyotonic run