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Properties of the TRPML3 Channel Pore and Its Stable Expansion by the Varitint-Waddler-causing Mutation
DC Field | Value | Language |
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dc.contributor.author | Kim, Hyun Jin | - |
dc.contributor.author | Yamaguchi, Soichiro | - |
dc.contributor.author | Li, Qin | - |
dc.contributor.author | So, Insuk | - |
dc.contributor.author | Muallem, Shmuel | - |
dc.date.accessioned | 2012-05-22T05:01:23Z | - |
dc.date.available | 2012-05-22T05:01:23Z | - |
dc.date.issued | 2010-05-28 | - |
dc.identifier.citation | JOURNAL OF BIOLOGICAL CHEMISTRY; Vol.285 22; 16513-16520 | ko_KR |
dc.identifier.issn | 0021-9258 | - |
dc.identifier.uri | https://hdl.handle.net/10371/76224 | - |
dc.description.abstract | TRPML3 is a H(+)-regulated Ca(2+) channel that shuttles between intracellular compartments and the plasma membrane. The A419P mutation causes the varitint-waddler phenotype as a result of gain-of-function (GOF). The mechanism by which A419P leads to GOF is not known. Here, we show that the TRPML3 pore is dynamic when conducting Ca(2+) to change its conductance and permeability, which appears to be mediated by trapping Ca(2+) within the pore. The pore properties can be restored by strong depolarization or by conducting Na(+) through the pore. The A419P mutation results in expanded channel pore with altered permeability that limits modulation of the pore by Ca(2+). This effect is specific for the A419P mutation and is not reproduced by other GOF mutations, including A419G, H283A, and proline mutations in the fifth transmembrane domain. These findings describe a novel mode of a transient receptor potential channel behavior and suggest that pore expansion by the A419P mutation may contribute to the varitint-waddler phenotype. | ko_KR |
dc.language.iso | en | ko_KR |
dc.publisher | AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC | ko_KR |
dc.title | Properties of the TRPML3 Channel Pore and Its Stable Expansion by the Varitint-Waddler-causing Mutation | ko_KR |
dc.type | Article | ko_KR |
dc.contributor.AlternativeAuthor | 김현진 | - |
dc.contributor.AlternativeAuthor | 소인석 | - |
dc.identifier.doi | 10.1074/jbc.M109.078204 | - |
dc.citation.journaltitle | JOURNAL OF BIOLOGICAL CHEMISTRY | - |
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dc.description.tc | 4 | - |
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