ClC-2 | ClC family | IUPHAR/BPS Guide to PHARMACOLOGY

ClC-2

Target id: 699

Nomenclature: ClC-2

Family: ClC family

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

   GtoImmuPdb view: OFF :     Currently no data for ClC-2 in GtoImmuPdb

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human 10 898 3q26-qter CLCN2 chloride voltage-gated channel 2
Mouse 10 908 16 B1 Clcn2 chloride channel, voltage-sensitive 2
Rat 10 907 11q23 Clcn2 chloride voltage-gated channel 2
Previous and Unofficial Names
CLC2 | chloride channel 2 | EJM6 | chloride channel, voltage-sensitive 2 | chloride channel
Database Links
CATH/Gene3D
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Gene
OMIM
Orphanet
UniProtKB
Wikipedia
Functional Characteristics
γ = 2–3 pS; voltage-activated by membrane hyperpolarization by fast protopore and slow cooperative gating; channels only open negative to ECl resulting in steady-state inward rectification; voltage dependence modulated by permeant anions; activated by cell swelling, PKA, and weak extracellular acidosis; potentiated by SGK1; inhibited by phosphorylation by p34(cdc2)/cyclin B; cell surface expression and activity increased by association with Hsp90

Download all structure-activity data for this target as a CSV file

Activators
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Units Concentration range (M) Voltage-dependent (mV) Reference
arachidonic acid Hs - - - - no

Not voltage dependent
lubiprostone Hs - - - - no

Not voltage dependent
omeprazole Hs - - - - no

Not voltage dependent
Activator Comments
CIC-2 is also activated by amidation.
Channel Blockers
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Units Concentration range (M) Voltage-dependent (mV) Reference
GaTx2 Hs - 10.8 pKd - yes (-100.0)
pKd 10.8 (Kd 1.5x10-11 M)
Voltage dependent: -100.0 mV
Zn2+ Hs - - - - no

Not voltage dependent
Cd2+ Hs - - - - no

Not voltage dependent
diphenylamine-2-carboxylic acid Hs - - - - no

Not voltage dependent
NPPB Hs - - - - no

Not voltage dependent
Clinically-Relevant Mutations and Pathophysiology
Disease:  Epilepsy, idiopathic generalized, susceptibility to, 11; EIG11
Synonyms: Idiopathic generalized epilepsy [Disease Ontology: DOID:1827]
Disease Ontology: DOID:1827
OMIM: 607628
Disease:  Epilepsy, juvenile myoclonic, susceptibility to, 8; EJM8
Synonyms: Juvenile myoclonic epilepsy [Orphanet: ORPHA307] [Disease Ontology: DOID:4890]
Disease Ontology: DOID:4890
OMIM: 607628
Orphanet: ORPHA307
Disease:  Leukoencephalopathy with mild cerebellar ataxia and white matter edema
OMIM: 615651
Orphanet: ORPHA363540
References:  1

References

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1. Depienne C, Bugiani M, Dupuits C, Galanaud D, Touitou V, Postma N, van Berkel C, Polder E, Tollard E, Darios F et al.. (2013) Brain white matter oedema due to ClC-2 chloride channel deficiency: an observational analytical study. Lancet Neurol, 12 (7): 659-68. [PMID:23707145]

How to cite this page

ClC family: ClC-2. Last modified on 23/02/2015. Accessed on 18/11/2018. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=699.