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| Content Provider | Springer Nature Link |
|---|---|
| Author | Klugbauer, rbert Marais, Else Hofmann, Franz |
| Copyright Year | 2003 |
| Abstract | Voltage-activated calcium channels are transmembrane proteins that act as transducers of electrical signals into numerous intracellular activities. On the basis of their electrophysiological properties they are classified as high- and low-voltage-activated calcium channels. High-voltage-activated calcium channels are heterooligomeric proteins consisting of a pore-forming α$_{1}$ subunit and auxiliary α$_{2}$δ, β, and—in some tissues—γ subunits. Auxiliary subunits support the membrane trafficking of the α$_{1}$ subunit and modulate the kinetic properties of the channel. In particular, the α$_{2}$δ subunit has been shown to modify the biophysical and pharmacological properties of the α$_{1}$ subunit. The α$_{2}$δ subunit is posttranslationally cleaved to form disulfide-linked α$_{2}$ and, δ proteins, both of which are heavily glycosylated. Recently it was shown that at least four genes encode for α$_{2}$δ subunits which are expressed in a tissue-specific manner. Their biophysical properties were characterized in coexpression studies with high- and low-voltage-activated calcium channels. Mutations in the gene encoding α$_{2}$δ-2 have been found to underlie the ducky phenotype. This mouse mutant is a model for absence epilepsy and is characterized by spike wave seizures and cerebellar ataxia. α$_{2}$δ subunits can also support pharmacological interactions with drugs that are used for the treatment of epilepsy and neuropathic pain. |
| Starting Page | 639 |
| Ending Page | 647 |
| Page Count | 9 |
| File Format | |
| ISSN | 0145479X |
| Journal | Journal of Bioenergetics and Biomembranes |
| Volume Number | 35 |
| Issue Number | 6 |
| e-ISSN | 15736881 |
| Language | English |
| Publisher | Kluwer Academic Publishers-Plenum Publishers |
| Publisher Date | 2003-01-01 |
| Publisher Place | New York |
| Access Restriction | One Nation One Subscription (ONOS) |
| Subject Keyword | Organic Chemistry Bioorganic Chemistry Biochemistry Animal Biochemistry Animal Anatomy / Morphology / Histology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Cell Biology Physiology |
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