{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T03:16:51Z","timestamp":1791343011600,"version":"4.3.1"},"reference-count":50,"publisher":"Society for Neuroscience","issue":"17","license":[{"start":{"date-parts":[[2008,10,23]],"date-time":"2008-10-23T00:00:00Z","timestamp":1224720000000},"content-version":"vor","delay-in-days":183,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Neurosci."],"published-print":{"date-parts":[[2008,4,23]]},"abstract":"<jats:p>\n                    Channelopathies are often linked to defective protein folding and trafficking. Among them, the calcium channelopathy episodic ataxia type-2 (EA2) is an autosomal dominant disorder related to mutations in the pore-forming Ca\n                    <jats:sub>v<\/jats:sub>\n                    2.1 subunit of P\/Q-type calcium channels. Although EA2 is linked to loss of Ca\n                    <jats:sub>v<\/jats:sub>\n                    2.1 channel activity, the molecular mechanism underlying dominant inheritance remains unclear. Here, we show that EA2 mutants as well as a truncated form (D\n                    <jats:sub>I-II<\/jats:sub>\n                    ) of the Ca\n                    <jats:sub>v<\/jats:sub>\n                    3.2 subunit of T-type calcium channel are misfolded, retained in the endoplasmic reticulum, and subject to proteasomal degradation. Pulse-chase experiments revealed that misfolded mutants bind to nascent wild-type Ca\n                    <jats:sub>v<\/jats:sub>\n                    subunits and induce their subsequent degradation, thereby abolishing channel activity. We conclude that this destructive interaction mechanism promoted by Ca\n                    <jats:sub>v<\/jats:sub>\n                    mutants is likely to occur in EA2 and in other inherited dominant channelopathies.\n                  <\/jats:p>","DOI":"10.1523\/jneurosci.2844-07.2008","type":"journal-article","created":{"date-parts":[[2008,4,23]],"date-time":"2008-04-23T12:56:20Z","timestamp":1208955380000},"page":"4501-4511","source":"Crossref","is-referenced-by-count":75,"title":["A Destructive Interaction Mechanism Accounts for Dominant-Negative Effects of Misfolded Mutants of Voltage-Gated Calcium 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