Novel brain expression of ClC-1 chloride channels and enrichment of CLCN1 variants in epilepsy

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Novel brain expression of ClC-1 chloride channels and enrichment of CLCN1 variants in epilepsy.

OBJECTIVE To explore the potential contribution of genetic variation in voltage-gated chloride channels to epilepsy, we analyzed CLCN family (CLCN1-7) gene variant profiles in individuals with complex idiopathic epilepsy syndromes and determined the expression of these channels in human and murine brain. METHODS We used parallel exomic sequencing of 237 ion channel subunit genes to screen ind...

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The skeletal muscle chloride channel CLC-1 and the ubiquitous volume-activated chloride channel CLC-2 belong to a large gene family whose members often show overlapping expression patterns. CLC-1 and CLC-2 are coexpressed in skeletal and smooth muscle and in the heart. By coexpressing CLC-1 and CLC-2 in Xenopus oocytes, we now show the formation of novel CLC-1/CLC-2 heterooligomers that yield t...

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CLC proteins are found in cells from prokaryotes to mammals and perform functions in plasma membranes and intracellular vesicles. Several genetic human diseases and mouse models underscore their broad physiological functions in mammals. These functions range from the control of excitability to transepithelial transport, endocytotic trafficking and acidification of synaptic vesicles. The recent ...

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Into ion channel and transporter function. Caenorhabditis elegans ClC-type chloride channels: novel variants and functional expression.

Six ClC-type chloride channel genes have been identified in Caenorhabditis elegans, termed clh-1 through clh-6. cDNA sequences from these genes suggest that clh-2, clh-3, and clh-4 may code for multiple channel variants, bringing the total to at least nine channel types in this nematode. Promoter-driven green fluorescent protein (GFP) expression in transgenic animals indicates that the protein ...

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ژورنال

عنوان ژورنال: Neurology

سال: 2013

ISSN: 0028-3878,1526-632X

DOI: 10.1212/wnl.0b013e31828868e7