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Chloride channel protein 2 (ClC-2)
- Target
- ClC-2
- Molecular classification
- Ion channel, ClC family of voltage-gated chloride channels
01
Overview
ClC-2 is a voltage-gated chloride channel protein that belongs to the ClC family. It is a homodimeric, double-barreled channel found in various tissues, including the brain, kidney, and gastrointestinal tract. ClC-2 plays a crucial role in regulating electrical excitability, maintaining ion homeostasis, and facilitating transepithelial transport. Its activity is modulated by intracellular chloride concentration, pH, and interacting proteins like GlialCAM. Disruptions or mutations in CLCN2 have been linked to leukoencephalopathy and epilepsy.
- Other names
- CLCN2Chloride channel protein type II
02
Mechanism of action
Modulation of chloride ion conductance across cell membranes via hyperpolarization-gated channel activity.
03
Biological functions
Regulation of electrical excitabilityMaintenance of extra-/intracellular ion homeostasisTransepithelial transportEpithelial barrier functionVolume regulation
04
Disease associations
LeukoencephalopathyEpilepsyNeurological disorders
05
Safety considerations
Disruption can lead to leukodystrophy-like syndromes.Loss-of-function mutations associated with epilepsy.
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