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Article

Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528H.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 15 Dec 1998

Morrill J A, Brown R H, Cannon S C

Abstract excerpt

The skeletal muscle L-type Ca channel serves a dual role as a calcium-conducting pore and as the voltage sensor coupling t-tubule depolarization to calcium release from the sarcoplasmic reticulum. Mutations in this channel cause hypokalemic periodic paralysis (HypoPP), a human autosomal dominant disorder characterized by episodic failure of muscle excitability that occurs in association with a decrease in serum...

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Identifiers

PMID: 9852570
PMCID: PMC6793372

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528H.PMID 9852570Read ↗

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Expression and functional characterization of the cardiac L-type calcium channel carrying a skeletal muscle DHP-receptor mutation causing hypokalaemic periodic paralysis.Semantic proximity Read ↗

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Hypokalemic periodic paralysis: an autosomal dominant muscle disorder caused by mutations in a voltage-gated calcium channel.Semantic proximity Read ↗

Channelopathies of skeletal muscle excitability.Semantic proximity Read ↗

Voltage-Dependent Ca <sup>2+</sup> Release Is Impaired in Hypokalemic Periodic Paralysis Caused by Ca <sub>V</sub> 1.1-R528H but not by Na <sub>V</sub> 1.4-R669HSemantic proximity Read ↗

A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore.Semantic proximity Read ↗

NaV1.4 DI-S4 periodic paralysis mutation R222W enhances inactivation and promotes leak current to attenuate action potentials and depolarize muscle fibers.Semantic proximity Read ↗

Channelopathies: potassium-related periodic paralyses and similar disorders.Semantic proximity Read ↗

Stac3 enhances expression of human CaV1.1 in Xenopus oocytes and reveals gating pore currents in HypoPP mutant channelsSemantic proximity Read ↗

Skeletal muscle DHP receptor mutations alter calcium currents in human hypokalaemic periodic paralysis myotubes.Semantic proximity Read ↗

Effects of mutations causing hypokalaemic periodic paralysis on the skeletal muscle L-type Ca2+ channel expressed in Xenopus laevis oocytes.Semantic proximity Read ↗

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