Leukoencephalopathy upon disruption of the chloride channel ClC-2.

doi:10.1523/JNEUROSCI.0338-07.2007

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Article

Leukoencephalopathy upon disruption of the chloride channel ClC-2.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 13 Jun 2007

Blanz Judith, Schweizer Michaela, Auberson Muriel, Maier Hannes, Muenscher Adrian, Hübner Christian A, Jentsch Thomas J

Abstract excerpt

ClC-2 is a broadly expressed plasma membrane chloride channel that is modulated by voltage, cell swelling, and pH. A human mutation leading to a heterozygous loss of ClC-2 has previously been reported to be associated with epilepsy, whereas the disruption of Clcn2 in mice led to testicular and retinal degeneration. We now show that the white matter of the brain and spinal cord of ClC-2 knock-out mice developed...

Topics

Age Factors
Animals
Axons
Blood-Brain Barrier
Brain
Brain Diseases
CLC-2 Chloride Channels
Canavan Disease
Chloride Channels
Demyelinating Diseases
Epilepsy

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Glutamic Acid

Mice

Mice, Knockout

Microscopy, Electron, Transmission

Mutation

Myelin Sheath

RNA, Complementary

Source

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Identifiers

PMID: 17567819
DOI: 10.1523/JNEUROSCI.0338-07.2007
PMCID: PMC6672451

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