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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
2
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pubmed:dateCreated |
2001-3-6
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pubmed:abstractText |
The subthreshold, voltage-gated potassium channel of skeletal muscle is shown to contain MinK-related peptide 2 (MiRP2) and the pore-forming subunit Kv3.4. MiRP2-Kv3.4 channels differ from Kv3.4 channels in unitary conductance, voltage-dependent activation, recovery from inactivation, steady-state open probability, and block by a peptide toxin. Thus, MiRP2-Kv3.4 channels set resting membrane potential (RMP) and do not produce afterhyperpolarization or cumulative inactivation to limit action potential frequency. A missense mutation is identified in the gene for MiRP2 (KCNE3) in two families with periodic paralysis and found to segregate with the disease. Mutant MiRP2-Kv3.4 complexes exhibit reduced current density and diminished capacity to set RMP. Thus, MiRP2 operates with a classical potassium channel subunit to govern skeletal muscle function and pathophysiology.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/BDS II protein, Anemonia sulcata,
http://linkedlifedata.com/resource/pubmed/chemical/Cnidarian Venoms,
http://linkedlifedata.com/resource/pubmed/chemical/KCNE3 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Kcnc4 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Kv3.4 protein, Xenopus,
http://linkedlifedata.com/resource/pubmed/chemical/Kv3.4 protein, rat,
http://linkedlifedata.com/resource/pubmed/chemical/Potassium Channels,
http://linkedlifedata.com/resource/pubmed/chemical/Potassium Channels, Voltage-Gated,
http://linkedlifedata.com/resource/pubmed/chemical/Protein Subunits,
http://linkedlifedata.com/resource/pubmed/chemical/Shaw Potassium Channels,
http://linkedlifedata.com/resource/pubmed/chemical/Xenopus Proteins
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pubmed:status |
MEDLINE
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pubmed:month |
Jan
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pubmed:issn |
0092-8674
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
26
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pubmed:volume |
104
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
217-31
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:11207363-Animals,
pubmed-meshheading:11207363-Cell Line,
pubmed-meshheading:11207363-Cell Membrane,
pubmed-meshheading:11207363-Chromosome Mapping,
pubmed-meshheading:11207363-Chromosomes, Human, Pair 11,
pubmed-meshheading:11207363-Cnidarian Venoms,
pubmed-meshheading:11207363-Cricetinae,
pubmed-meshheading:11207363-Electrophysiology,
pubmed-meshheading:11207363-Female,
pubmed-meshheading:11207363-Humans,
pubmed-meshheading:11207363-Immunohistochemistry,
pubmed-meshheading:11207363-Male,
pubmed-meshheading:11207363-Membrane Potentials,
pubmed-meshheading:11207363-Mice,
pubmed-meshheading:11207363-Muscle, Skeletal,
pubmed-meshheading:11207363-Mutation, Missense,
pubmed-meshheading:11207363-Oocytes,
pubmed-meshheading:11207363-Paralyses, Familial Periodic,
pubmed-meshheading:11207363-Patch-Clamp Techniques,
pubmed-meshheading:11207363-Pedigree,
pubmed-meshheading:11207363-Potassium Channels,
pubmed-meshheading:11207363-Potassium Channels, Voltage-Gated,
pubmed-meshheading:11207363-Protein Subunits,
pubmed-meshheading:11207363-Rats,
pubmed-meshheading:11207363-Shaw Potassium Channels,
pubmed-meshheading:11207363-Xenopus Proteins,
pubmed-meshheading:11207363-Xenopus laevis
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pubmed:year |
2001
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pubmed:articleTitle |
MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis.
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pubmed:affiliation |
Departments of Pediatrics and Cellular, Molecular Physiology, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, CT 06536, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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