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J. Biol. Chem., Vol. 276, Issue 51, 48077-48082, December 21, 2001
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From the Malignant hyperthemia (MH) is a pharmacogenetic
disease triggered by volatile anesthetics and succinylcholine in
genetically predisposed individuals. The underlying feature of MH is a
hypersensitivity of the calcium release machinery of the sarcoplasmic
reticulum, and in many cases this is a result of point mutations in the
skeletal muscle ryanodine receptor calcium release channel (RYR1). RYR1 is mainly expressed in skeletal muscle, but a recent report
demonstrated the existence of this isoform in human B-lymphocytes. As
B-cells can produce a number of cytokines, including endogenous
pyrogens, we investigated whether some of the symptoms seen during MH
could be related to the involvement of the immune system. Our results show that (i) Epstein-Barr virus-immortalized B-cells from
MH-susceptible individuals carrying the V2168M RYR1 gene mutation were
more sensitive to the RYR activator 4-chloro-m-cresol and
(ii) their peripheral blood leukocytes produce more interleukin
(IL)-1
B-lymphocytes from Malignant Hyperthermia-susceptible Patients
Have an Increased Sensitivity to Skeletal Muscle Ryanodine Receptor
Activators*
,,§, and
Departments of Anaesthesia and Research,
Hebelstrasse 20, University of Basel Kantonsspital, 4031 Basel,
Switzerland, the § Department of Experimental and Diagnostic
Medicine, Section of General Pathology, University of Ferrara, Via
Borsari 46, 44100 Ferrara, Italy, and the ¶ Department of Surgery,
Division of Research, Hebelstrasse 20, University of Basel
Kantonsspital, 4031 Basel, Switzerland
after treatment with the RYR activators caffeine and
4-chloro-m-cresol, compared with cells from healthy
controls. Our result demonstrate that RYR1-mediated calcium signaling
is involved in release of IL-1 from B-lymphocytes and suggest that
some of the symptoms seen during an MH episode may be due to
IL-1 production.
*
This work was supported in part by Swiss National Foundation
Grant 3200-063959.00, Telethon Italy Grant 1259 (to F. Z.), a grant from the Ministero Università e Ricerca Scientifica e
Tecnologica ex 40%, and by the Department of Anesthesia, Basel
Kantonsspital.The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in
accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
To whom correspondence should be addressed: ZLF Basel
Kantonsspital, Laboratory 408, Hebelstr. 20, 4031 Basel,
Switzerland. Tel.: 41-61-265-2373; Fax: 41-61-265-3702; E-mail:
susan.treves@unibas.ch.
Copyright © 2001 by The American Society for Biochemistry and Molecular Biology, Inc.
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