Pharmacologic and functional characterization of malignant hyperthermia in the R163C RyR1 knock-in mouse.
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Malignant hyperthermiaPharmGKB summary: succinylcholine pathway, pharmacokinetics/pharmacodynamicsMalignant hyperthermia: a reviewCa(V)1.1: The atypical prototypical voltage-gated Ca²⁺ channelDivergent Activity Profiles of Type 1 Ryanodine Receptor Channels Carrying Malignant Hyperthermia and Central Core Disease Mutations in the Amino-Terminal RegionAnesthetic- and heat-induced sudden death in calsequestrin-1-knockout miceMyoplasmic resting Ca2+ regulation by ryanodine receptors is under the control of a novel Ca2+-binding region of the receptor.RyR1-mediated Ca2+ leak and Ca2+ entry determine resting intracellular Ca2+ in skeletal myotubes.Ca2+ influx via the Na+/Ca2+ exchanger is enhanced in malignant hyperthermia skeletal muscle.A malignant hyperthermia-inducing mutation in RYR1 (R163C): alterations in Ca2+ entry, release, and retrograde signaling to the DHPR.A malignant hyperthermia-inducing mutation in RYR1 (R163C): consequent alterations in the functional properties of DHPR channelsDantrolene mitigates caerulein-induced pancreatitis in vivo in mice.Malignant hyperthermia susceptibility arising from altered resting coupling between the skeletal muscle L-type Ca2+ channel and the type 1 ryanodine receptorMuscle weakness in Ryr1I4895T/WT knock-in mice as a result of reduced ryanodine receptor Ca2+ ion permeation and release from the sarcoplasmic reticulum.Basal bioenergetic abnormalities in skeletal muscle from ryanodine receptor malignant hyperthermia-susceptible R163C knock-in mice.Functional and biochemical properties of ryanodine receptor type 1 channels from heterozygous R163C malignant hyperthermia-susceptible mice.Malignant hyperthermia: a pharmacogenetic disorder.Accelerated activation of SOCE current in myotubes from two mouse models of anesthetic- and heat-induced sudden death.Gene dose influences cellular and calcium channel dysregulation in heterozygous and homozygous T4826I-RYR1 malignant hyperthermia-susceptible muscle.Mice expressing T4826I-RYR1 are viable but exhibit sex- and genotype-dependent susceptibility to malignant hyperthermia and muscle damageAntioxidants protect calsequestrin-1 knockout mice from halothane- and heat-induced sudden death.Nonspecific sarcolemmal cation channels are critical for the pathogenesis of malignant hyperthermia.Halothane modulation of skeletal muscle ryanodine receptors: dependence on Ca2+, Mg2+, and ATP.Genetically manipulated mice: a powerful tool with unsuspected caveats.Enhanced excitation-coupled calcium entry in myotubes expressing malignant hyperthermia mutation R163C is attenuated by dantrolene.Ca2+ dysregulation in Ryr1(I4895T/wt) mice causes congenital myopathy with progressive formation of minicores, cores, and nemaline rodsCalsequestrin-1: a new candidate gene for malignant hyperthermia and exertional/environmental heat stroke.Ryanodine receptor studies using genetically engineered mice.The disorders of the calcium release unit of skeletal muscles: what have we learned from mouse models?A guide to the 3D structure of the ryanodine receptor type 1 by cryoEM.Genotype-Phenotype Correlations of Malignant Hyperthermia and Central Core Disease Mutations in the Central Region of the RYR1 Channel.Altered Ca2+ homeostasis in the skeletal muscle of DJ-1 null mice.Ryanodine receptor type 1 (RyR1) possessing malignant hyperthermia mutation R615C exhibits heightened sensitivity to dysregulation by non-coplanar 2,2',3,5',6-pentachlorobiphenyl (PCB 95).Successful management of malignant hyperpyrexia syndrome in a pediatric patient: What to do when dantrolene is not available?Estrogens Protect Calsequestrin-1 Knockout Mice from Lethal Hyperthermic Episodes by Reducing Oxidative Stress in Muscle.Altered Ca2+ concentration, permeability and buffering in the myofibre Ca2+ store of a mouse model of malignant hyperthermia.Voltage modulates halothane-triggered Ca2+ release in malignant hyperthermia-susceptible muscle.Malignant Hyperthermia in the Post-Genomics Era: New Perspectives on an Old Concept.Strenuous exercise triggers a life-threatening response in mice susceptible to malignant hyperthermia.Reduced threshold for store overload-induced Ca2+ release is a common defect of RyR1 mutations associated with malignant hyperthermia and central core disease.
P2860
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P2860
Pharmacologic and functional characterization of malignant hyperthermia in the R163C RyR1 knock-in mouse.
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2006 nî lūn-bûn
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2006 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի դեկտեմբերին հրատարակված գիտական հոդված
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2006年の論文
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2006年論文
@yue
2006年論文
@zh-hant
2006年論文
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2006年論文
@zh-mo
2006年論文
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2006年论文
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name
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@ast
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@en
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@nl
type
label
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@ast
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@en
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@nl
prefLabel
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@ast
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@en
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@nl
P2093
P1433
P1476
Pharmacologic and functional c ...... the R163C RyR1 knock-in mouse.
@en
P2093
Eric Esteve
Isaac N Pessah
José R Lopez
Joyce Riehl
Klaus I Matthaei
Paul D Allen
Samuel Goth
Tianzhong Yang
P304
P356
10.1097/00000542-200612000-00016
P407
P577
2006-12-01T00:00:00Z