| pubmed-article:16527851 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0086418 | lld:lifeskim |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0001675 | lld:lifeskim |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0034792 | lld:lifeskim |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0031164 | lld:lifeskim |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0596235 | lld:lifeskim |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0439799 | lld:lifeskim |
| pubmed-article:16527851 | lifeskim:mentions | umls-concept:C0449560 | lld:lifeskim |
| pubmed-article:16527851 | pubmed:issue | Pt 1 | lld:pubmed |
| pubmed-article:16527851 | pubmed:dateCreated | 2006-5-16 | lld:pubmed |
| pubmed-article:16527851 | pubmed:abstractText | Slow-channel congenital myasthenic syndrome, caused by mutations in subunits of the endplate ACh receptor (AChR), results in prolonged synaptic currents and excitotoxic injury of the postsynaptic region by Ca2+ overloading. The Ca2+ overloading could be due entirely to the prolonged openings of the AChR channel or could be abetted by enhanced Ca2+ permeability of the mutant channels. We therefore measured the fractional Ca2+ current, defined as the percentage of the total ACh-evoked current carried by Ca2+ ions (Pf), for AChRs harbouring the alphaG153S or the alphaV249F slow-channel mutation, and for wild-type human AChRs in which Pf has not yet been determined. Experiments were performed in transiently transfected GH4C1 cells and human myotubes with simultaneous recording of ACh-evoked whole-cell currents and fura-2 fluorescence signals. We found that the Pf of the wild-type human endplate AChR was unexpectedly high (Pf approximately 7%), but neither the alphaV249F nor the alphaG153S mutation altered Pf. Fetal human AChRs containing either the wild-type or the mutated alpha subunit had a much lower Pf (2-3%). We conclude that the Ca2+ permeability of human endplate AChRs is higher than that reported for any other human nicotinic AChR, with the exception of alpha7-containing AChRs (Pf > 10%); and that neither the alphaG153S nor the alphaV249F mutations affect the Pf of fetal or adult endplate AChRs. However, the intrinsically high Ca2+ permeability of human AChRs probably predisposes to development of the endplate myopathy when opening events of the AChR channel are prolonged by altered AChR-channel kinetics. | lld:pubmed |
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| pubmed-article:16527851 | pubmed:language | eng | lld:pubmed |
| pubmed-article:16527851 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16527851 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:16527851 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:16527851 | pubmed:month | May | lld:pubmed |
| pubmed-article:16527851 | pubmed:issn | 0022-3751 | lld:pubmed |
| pubmed-article:16527851 | pubmed:author | pubmed-author:EngelAndrew... | lld:pubmed |
| pubmed-article:16527851 | pubmed:author | pubmed-author:FucileSergioS | lld:pubmed |
| pubmed-article:16527851 | pubmed:author | pubmed-author:EusebiFabrizi... | lld:pubmed |
| pubmed-article:16527851 | pubmed:author | pubmed-author:GrassiFrances... | lld:pubmed |
| pubmed-article:16527851 | pubmed:author | pubmed-author:SucapaneAnton... | lld:pubmed |
| pubmed-article:16527851 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:16527851 | pubmed:day | 15 | lld:pubmed |
| pubmed-article:16527851 | pubmed:volume | 573 | lld:pubmed |
| pubmed-article:16527851 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:16527851 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:16527851 | pubmed:pagination | 35-43 | lld:pubmed |
| pubmed-article:16527851 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:16527851 | pubmed:year | 2006 | lld:pubmed |
| pubmed-article:16527851 | pubmed:articleTitle | The human adult subtype ACh receptor channel has high Ca2+ permeability and predisposes to endplate Ca2+ overloading. | lld:pubmed |
| pubmed-article:16527851 | pubmed:affiliation | Pasteur Institute -Cenci Bolognetti Foundation & Department of Human Physiology and Pharmacology & Centre of Excellence for Biology and Molecular Medicine, University of Rome La Sapienza, Piazzale Aldo Moro 5; I-00185 Rome, Italy. | lld:pubmed |
| pubmed-article:16527851 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:16527851 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| pubmed-article:16527851 | pubmed:publicationType | Research Support, N.I.H., Extramural | lld:pubmed |
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