3,5-Di-t-butyl catechol (DTCAT) as an activator of the human skeletal muscle ryanodine receptor Ca2+ channel and its evaluation as a test substance for the assessment of susceptibility to malignant hyperthermia
نویسندگان
چکیده
Open Access Meeting abstract 3,5-Di-t-butyl catechol (DTCAT) as an activator of the human skeletal muscle ryanodine receptor Ca2+ channel and its evaluation as a test substance for the assessment of susceptibility to malignant hyperthermia Caterina Lacava2, Andrea Michalek-Sauberer1, Birgit Kraft1, Giampietro Sgaragli2, Elisabeth Sipos1, Carmen Höller1, Hans-Georg Kress1, Fabio Fusi2 and Lukas G Weigl*1
منابع مشابه
Functional characterization of a distinct ryanodine receptor mutation in human malignant hyperthermia-susceptible muscle.
Malignant hyperthermia is an inherited autosomal disorder of skeletal muscle in which certain volatile anesthetics and depolarizing muscle relaxants trigger an abnormally high release of Ca2+ from the intracellular Ca2+ store, the sarcoplasmic reticulum. In about 50% of cases, malignant hyperthermia susceptibility is linked to the gene encoding the skeletal muscle ryanodine receptor/Ca2+ releas...
متن کاملFunctional analysis of RYR1 variants linked to malignant hyperthermia
Malignant hyperthermia manifests as a rapid and sustained rise in temperature in response to pharmacological triggering agents, e.g. inhalational anesthetics and the muscle relaxant suxamethonium. Other clinical signs include an increase in end-tidal CO2, increased O2 consumption, as well as tachycardia, and if untreated a malignant hyperthermia episode can result in death. The metabolic change...
متن کاملIncreased sensitivity of the ryanodine receptor to halothane-induced oligomerization in malignant hyperthermia-susceptible human skeletal muscle.
Mutations in the skeletal muscle RyR1 isoform of the ryanodine receptor (RyR) Ca2+-release channel confer susceptibility to malignant hyperthermia, which may be triggered by inhalational anesthetics such as halothane. Using immunoblotting, we show here that the ryanodine receptor, calmodulin, junctin, calsequestrin, sarcalumenin, calreticulin, annexin-VI, sarco(endo)plasmic reticulum Ca2+-ATPas...
متن کاملSkeletal muscle ryanodine receptor mutations associated with malignant hyperthermia showed enhanced intensity and sensitivity to triggering drugs when expressed in human embryonic kidney cells.
BACKGROUND Mutations within the gene encoding the skeletal muscle calcium channel ryanodine receptor can result in malignant hyperthermia. Although it is important to characterize the functional effects of candidate mutations to establish a genetic test for diagnosis, ex vivo methods are limited because of the low incidence of the disorder and sample unavailability. More than 250 candidate muta...
متن کامل4-Chloro-m-cresol, a potent and specific activator of the skeletal muscle ryanodine receptor.
The aim of the present study was to determine the effects of 4-chloro-m-cresol (4-CmC), a preservative often added to drugs intravenously administered, on the skeletal muscle sarcoplasmic reticulum (SR) Ca2+ release channel/ryanodine receptor. In heavy SR vesicles obtained from rabbit back muscles, 4-CmC stimulated (Ca2+)-activated [3H]ryanodine binding with an EC50 of about 100 microM. In the ...
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عنوان ژورنال:
دوره 9 شماره
صفحات -
تاریخ انتشار 2009