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Hypermetabolism in B-lymphocytes from malignant hyperthermia susceptible individuals.

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Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle metabolism which is characterized by generalized muscle rigidity, increased body temperature, rhabdomyolysis, and severe metabolic acidosis. The underlying mechanism of MH involves excessive Ca(2+) release in myotubes via

Identification and Functional Analysis of RYR1 Variants in a Family with a Suspected Myopathy and Associated Malignant Hyperthermia.

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The ryanodine receptor 1 (RyR1) is a major skeletal muscle calcium release channel located in the sarcoplasmic reticulum and involved in excitation-contraction coupling. Variants in the gene encoding RyR1 have been linked to a range of neuromuscular disorders including myopathies and

Detection of proton release from cultured human myotubes to identify malignant hyperthermia susceptibility.

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BACKGROUND Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle. During general anesthesia, a life-threatening hypermetabolic state may occur resulting from increased release of Ca2+ from the sarcoplasmic reticulum in skeletal muscle. Diagnosis of MH susceptibility requires

4-chloro-m-cresol cannot detect malignant hyperthermia equivocal cells in an alternative minimally invasive diagnostic test of malignant hyperthermia susceptibility.

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Malignant hyperthermia (MH) is an inherited skeletal muscle disorder triggered by commonly used anesthetics. Mutated ryanodine receptors have been identified as molecular targets. The sensitivity of myotubes from individuals classified by the in vitro contracture test as MH susceptible (MHS), normal

A multicenter study of 4-chloro-m-cresol for diagnosing malignant hyperthermia susceptibility.

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Standardization of the in vitro contracture test (IVCT) for malignant hyperthermia (MH) susceptibility has resulted in very rare false negative tests. However, false positive results stigmatizing the patient seem to be more frequent than false negative results and make supplementary tests desirable.

4-Chloro-m-cresol: a specific tool to distinguish between malignant hyperthermia-susceptible and normal muscle.

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Single-channel recordings have indicated that ryanodine receptor (RyR1) mutation Arg615Cys of porcine malignant hyperthermia-susceptible (MHS) muscle is not directly associated with the enhanced caffeine sensitivity of MH(S) muscle [1]. In the present study, the effect of a novel activator of RyR1,

Classification of malignant hyperthermia-equivocal patients by 4-chloro-M-cresol.

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To clarify the contracture response to 4-chloro-m-cresol (4-CmC) in malignant hyperthermia (MH) equivocal (MHE) muscle, we studied the effect of cumulative concentrations of 4-CmC. In vitro contracture test (IVCT) was performed in 35 probands according to the European MH test protocol. Surplus

Characterization of ryanodine receptor-mediated calcium release in human B cells: relevance to diagnostic testing for malignant hyperthermia.

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BACKGROUND Mutations in the ryanodine type 1 receptor (RyR1) are causative for malignant hyperthermia. Studies in human B lymphocytes have shown that measurement of RyR1-mediated intracellular Ca(2+) (Ca(2+)(i)) release can differentiate between normal and malignant hyperthermia-susceptible

Multifunctional carboxymethyl cellulose-based magnetic nanovector as a theragnostic system for folate receptor targeted chemotherapy, imaging, and hyperthermia against cancer.

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A multifunctional biocompatible nanovector based on magnetic nanoparticle and carboxymethyl cellulose (CMC) was developed. The nanoparticles have been characterized using TEM, SEM, DLS, FT-IR spectra, VSM, and TGA studies. We found that the synthesized carboxymethyl cellulose magnetic nanoparticles

Multifunctional Polymeric Platform of Magnetic Ferrite Colloidal Superparticles for Luminescence, Imaging, and Hyperthermia Applications.

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Adequately designed multiresponsive water-soluble graft copolymers were used to serve as a multifunctional polymeric platform for the encapsulation and transfer in aqueous media of hydrophobic magnetic nanoparticles (MNPs). The backbone of the graft copolymers was composed of hydrophilic sodium

[4-chloro-m-cresol-induced contractures of skeletal muscle specimen from patients at risk for malignant hyperthermia].

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OBJECTIVE 4-chloro-m-cresol (4-CmC), commonly used as preservative, has been shown to induce contractures in skeletal muscle specimens from individuals susceptible to malignant hyperthermia (MH). It has been suggested that a defect of the calcium release channel of the skeletal muscle sarcoplasmic

Enhanced response to caffeine and 4-chloro-m-cresol in malignant hyperthermia-susceptible muscle is related in part to chronically elevated resting [Ca2+]i.

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Malignant hyperthermia (MH) is a potentially fatal pharmacogenetic syndrome caused by exposure to halogenated volatile anesthetics and/or depolarizing muscle relaxants. We have measured intracellular Ca(2+) concentration ([Ca(2+)](i)) using double-barreled, Ca(2+)-selective microelectrodes in

Effect of Norepinephrine on Intracellular Ca2+ Levels in Malignant Hyperthermia-Susceptible B Cells: Pilot Study in the Search for a New Diagnostic Test for Malignant Hyperthermia.

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Malignant hyperthermia (MH) crises may induce morbidity or death in MH-susceptible (MHS) individuals. The only sensitive method of determining susceptibility is the caffeine-halothane contracture test, requiring muscle biopsy. Early research on MH demonstrated an abnormal response to catecholamines

Cladribine in combination with mitoxantrone and cyclophosphamide(CMC) in the treatment of heavily pre-treated patients with advanced indolent lymphoid malignancies.

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The aim of our study was to determine the effectiveness and toxicity of combined chemotherapy consisting of cladribine (2-chloro-deoxyadenosine, 2-CdA), mitoxantrone and cyclophosphamide (CMC regimen) in the treatment of refractory or relapsed indolent lymphoproliferative disorders. The treatment

Biomimetic Mineralization of Tumor Targeted Ferromagnetic Iron Oxide Nanoparticles Used for Media of Magnetic Hyperthermia.

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BACKGROUND The magnetic hyperthermia has been recognized as a useful therapeutic modality for malignant tumors, and IONPs have received a great deal of attentions for potential biomedical applications. The aims of this paper are to design a biomimetic mineralization procedure to synthesize the

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