Structure and function of CLC Chloride Channels

Abstract

CLC voltage-gated chloride ion channels (and transporters) family is widely present in bacteria to humans from a variety of species, is responsible for regulation of chloride ion transmembrane anion transport. CLC channel proteins Jie Wei diamond homodimer each triangle monomer by 18 α-helix structure, has its own independent pore (pore) and selective filters (selectivity filter), and its internal symmetry was a double reverse parallel structures. CLC channels and transporter proteins ( ClC-0, -1, -2) of the gating mechanisms (gating) and infiltration of the mechanism (permeation) and chloride ion in the pore within the binding capacity (binding) closely related to the family show distinctive electrophysiological properties . its mammalian family members entrusted with regulation of muscle cell membrane excitability and movement in skin cells, transport, regulation of cell volume and the acidification of intracellular organelles such as the mission, so its function will lead to defects in myotonia, Bartter syndrome, Dent disease and bone Petrochemical springing a variety of genetic disease