Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seminal vesicle-specific antigen (SVSA) has been shown to be a polymorphic antigen represented by multiple immunoreactive peptides when fresh human semen is probed with monoclonal antibody (MHS-5) on Western blots. Semen samples collected directly into sodium dodecyl sulfate (SDS) demonstrate major immunoreactive peptide bands at 69-71 kDa and 58 kDa as well as a series of peptides of lower molecular mass. As semen liquefies, the higher molecular mass forms of SVSA are transformed into lower molecular mass bands, with 10-13 kDa immunoreactive peptides predominating after 8 h of liquefaction (McGee and Herr, Biol. Reprod. 37:431-439, 1987). In the present study, the 10-13 kDa form of SVSA was purified by preparative electrophoresis from SDS gels and a polyclonal antibody was generated in guinea pigs. Human seminal vesicle was fixed by immersion in combinations of glutaraldehyde and paraformaldehyde and embedded in Araldite or LR Gold. Both the guinea pig polyclonal antibody and the murine monoclonal antibody MHS-5 were employed to localize SVSA in human seminal vesicle by immunoelectron microscopy using Protein-A gold complexes. Gold particles were quantified in various subcellular compartments by a Videoplan computer. With either antibody probe, SVSA was found predominantly in the central electron-dense cores of secretory granules, with no staining evident over the electron lucent halo surrounding the granule core. With preimmune serum, the mean number of gold particles overlying secretory granules was 3/microns2; with polyclonal anti-SVSA, the mean number of particles observed over secretory granules was 182/microns2. This study represents, to our knowledge, the first fine-structural localization of a specific secretory protein to the electron-dense cores of secretory granules in principal cells of the human seminal vesicle.
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PMID:Electron microscopic immunolocalization of seminal vesicle-specific antigen in human seminal vesicle. 272 30

Previous studies have demonstrated that skeletal muscle from individuals susceptible to malignant hyperthermia (MH) has a defect associated with the mechanism of calcium release from its intracellular storage sites in the sarcoplasmic reticulum (SR). In this report we demonstrate that the [3H]ryanodine receptor of isolated MH-susceptible (MHS) porcine heavy SR exhibits an altered Ca2+ dependence of [3H]ryanodine binding at the low affinity Ca2+ site as well as a lower Kd for ryanodine (92 versus 265 nM) when compared to normal porcine SR. The Bmax of the normal and MHS [3H] ryanodine receptor (9.3-12.6 pmol/mg) was not significantly different, and analysis of MHS and normal SR proteins by sodium dodecyl sulfate-polyacrylamide gel electrophoresis did not reveal a significant difference in the intensity of Coomassie Blue staining of the spanning protein/ryanodine receptor region of the gels (Mr greater than 300,000). We also find that MHS porcine muscle intact fiber bundles exhibit a 5-10-fold lower ryanodine threshold for twitch and tetanus inhibition, and contracture onset when compared to normal muscle. Since the SR ryanodine receptor is a calcium release channel as well as a component intimately involved in transverse tubule-SR communication, abnormalities in the skeletal muscle ryanodine receptor may be responsible for the abnormal SR calcium release and contractile properties demonstrated by MHS muscle.
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PMID:Abnormal sarcoplasmic reticulum ryanodine receptor in malignant hyperthermia. 337 71

Binding of monoclonal antibody MHS-5 to western blots of human seminal plasma was employed to follow the fate of a seminal vesicle-specific antigen (SVSA) during semen liquefaction. Ejaculates from four vasectomized donors were collected in a manner to inhibit liquefaction or to allow liquefaction to proceed at room temperature. Aliquots of the liquefying seminal fluid were removed at specific time points and further liquefaction inhibited with sodium dodecyl sulfate (SDS). Western blot analysis using monoclonal antibody MHS-5 demonstrated that the SVSA epitope in all donors was located on multiple bands ranging in mass from 15-92 kDa in unliquefied ejaculates; immunoreactive peptides below 15 kDa were not detected. As early as 5 min post ejaculation, immunoreactive bands below 15 kDa were identified in liquefying samples. During the same time period (5 min), immunoreactive bands of 69-71 and 58 kDa could not be immunologically detected in liquefying samples. A decrease in immunoreactive staining of components higher molecular mass was accompanied by a concomitant increase in immunoreactive staining of intermediate and small molecular mass molecules during the first 2 h of liquefaction. After 8-24 h of liquefaction, two immunoreactive bands of 10.9 and 12.5 kDa predominated. Between 24 and 48 h, each donor's ejaculate demonstrated a common single immunoreactive band of 10.9 kDa. These results indicate that there is a rapid transformation in mass of the SVSA with major 69-71 and 58 kDa bands being converted to forms of lower mass within 5 min of ejaculation.
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PMID:Human seminal vesicle-specific antigen during semen liquefaction. 367 97

It has been suspected that the cause of malignant hyperthermia (MH) is an abnormality in the sarcoplasmic reticulum of skeletal muscle. We isolated the sarcoplasmic reticulum from malignant hyperthermia-susceptible (MHS) patients and controls and analysed the protein composition with sodium dodecyl sulfate polyacrylamide gel electrophoresis. There were no remarkable changes in the sarcoplasmic reticulum protein composition profile of the scanned gel of the patients. Quantitative measurement of the relative proportion of each band in the gel, however, revealed a slight decrease in calsequestrin and a slight increase in protein of molecular weight 23,000. (Ca2+ -Mg2+)ATPase had no altered subfragments in MHS patients. Crude mitochondrial proteins and myoplasmic proteins showed minor alterations in composition in some patients. The data supported the thesis that malignant hyperthermia is due to defects in several different cell membranes including the sarcoplasmic reticulum and the mitochondria.
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PMID:Analysis of sarcoplasmic reticulum proteins in patients susceptible to malignant hyperthermia. 687 11

Malignant hyperthermia is an autosomal dominant disorder with variable expressivity that is caused by a membrane defect in the sarcolemma of myofibrils. A patient with strabismus (esotropia) had tachycardia and masseter muscle rigidity on exposure to succinylcholine chloride and halothane, but because of rapid recognition of the condition and discontinuation of the procedure, the potentially lethal complications of malignant hyperthermia did not develop. A serum creatine phosphokinase level showed a substantial increase above normal. Two weeks later, the patient underwent successful correction of the strabismus under general anesthesia, using morphine sulfate and thiopental sodium without complication. This condition is of interest to ophthalmologists because it occurs with increasing frequency in patients with strabismus and ptosis, and it may be triggered by certain local anesthetic agents often used by ophthalmologists.
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PMID:Suspected malignant hyperthermia in a strabismus patient. A case report. 725 98

A case of suspected malignant hyperthermia in a 13-month-old female, to whom succinylcholine was not administered, is presented. The patient presented for a repair of the right radial nerve under general anesthesia. Induction was accomplished with halothane, nitrous oxide, and oxygen. Tracheal intubation was facilitated with intravenous vecuronium. Controlled ventilation was initiated, and anesthesia was maintained with isoflurane, nitrous oxide, oxygen, morphine sulfate, and vecuronium. At the conclusion of the surgical procedure, an abrupt increase in ETCO2, an elevation in body temperature, and a mixed acidosis was observed. Resolution of symptoms followed the administration of dantrolene sodium. The patient underwent an uneventful postoperative recovery and was discharged home. It was felt that the patient was too young to undergo a muscle biopsy for a caffeine-halothane stimulation test.
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PMID:Suspected malignant hyperthermia in a 13-month-old: today's "typical" episode--a case report. 923 94

Monolayers of the porcine-derived renal epithelial cell line, LLC-PK1, were used to characterize the effects of heat stress on Na+-glucose cotransport. Transepithelial current dependent on 5 mM glucose (I(Glc)), phloridzin-sensitive current (I(phz)), and total transepithelial current (I(total)) were measured as indicators of Na+-glucose cotransport. Severe heat shock (SHS; 45 degrees C for 1 h, then 37 degrees C for measurements) decreased transepithelial electrical resistance (TER), I(Glc), I(phz), and I(total) 50-70%. Mild heat shock (MHS; 42 degrees C for 3 h, then 37 degrees C for 12 h) induced accumulation of 72-kDa heat shock protein (HSP-72), decreased damage to TER from SHS, and prevented damage to I(Glc), I(phz), and I(total). Kinetic analysis showed that SHS damaged and MHS protected total Na+-glucose transport capacity (Vmax of I(Glc)). MHS alone increased TER (50%), I(Glc) (20%), I(total) (20%), and Vmax of I(Glc) (25%). On enhancement of the Na+ gradient by depletion of intracellular Na+, MHS increased I(Glc) 50% and had no effect on transepithelial Na+-dependent sulfate reabsorptive flux measured concurrently or in Na+-replete tissues. These effects of MHS were not reflected in effects on cell survival or luminal membrane surface area as indicated by lactate dehydrogenase or alkaline phosphatase release. In conclusion, HSP-72-inducing heat treatment both protected and enhanced Na+-glucose cotransport independently of the luminal membrane Na+ gradient and selectively with respect to effects on TER, reabsorptive sulfate transport, cell survival, and luminal membrane surface area.
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PMID:Heat shock-induced protection and enhancement of Na+-glucose cotransport by LLC-PK1 monolayers. 936 30

Acute asthma is well known to provoke complications. We report the case of a patient who needed intubation and mechanical ventilation for acute asthma. Despite a treatment with corticosteroids, bronchodilators, neuromuscular blocking drugs and magnesium sulfate, the situation remained uncontrolled and as a last resort, halothane became necessary. The patient then developed an episode of malignant hyperthermia with fever at 40 degrees C and rhabdomyolysis. At this time, halothane could be stopped and all the symptoms disappeared without modifying the rest of the treatment. Eight days later, he presented with a neuroleptic malignant syndrome following an injection of droperidol. Temperature rose to 42 degrees C, associated with muscle rigidity, sweating, tachycardia and severe circulatory collapse. The use of dantrolene in association with a symptomatic treatment of the collapse led to a favourable outcome in. Unfortunately, in vitro contracture test could not be performed in this case. The links between malignant hyperthermia and neuroleptic malignant syndrome remain unclear. Although these two pathologies share the same physiopathology, symptomatology and treatment, they are clearly individualized. This case seems to be the first description of their occurrence in the same patient.
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PMID:Malignant hyperthermia and neuroleptic malignant syndrome in a patient during treatment for acute asthma. 992

We gave propofol anesthesia to a patient with limb-girdle type of progressive muscular dystrophy. A 42 year-old male was to have skin graft for third degree burn. His respiratory function test showed %VC of 73.6% and %FEV1.0 of 107.6%. Arterial blood gas data were within normal ranges. He was anesthetized with propofol, fentanyl, vecuronium and nitrous oxide. During position change, Wenckebach type of second degree AV block occurred. AV block returned to sinus rhythm easily by injection of ephedrine hydrochloride and atropine sulfate, and reduction of propofol infusion rate. There were no perioperative respiratory complications and no clinical manifestations of malignant hyperthermia. Propofol anesthesia is suitable for limb-girdle type of progressive muscular dystrophy, because of very little possibility of triggering malignant hyperthermia, rapid awaking, minimal residual effects of the respiratory system, and easiness in controlling anesthetic depth.
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PMID:[Propofol anesthesia for a patient with progressive muscular dystrophy]. 1188 93

A new procedure for isolating and estimating ingested carbonaceous diesel exhaust particles (DEP) or carbon black (CB) particles by lung epithelial cells and macrophages is described. Cells were incubated with DEP or CB to examine cell-particle interaction and ingestion. After various incubation periods, the cells were separated from free extracellular DEP or CB particles by Ficoll density gradient centrifugation and dissolved in hot sodium dodecyl sulfate detergent. Insoluble DEP or CB residues were isolated by high-speed centrifugation, and the elemental carbon (EC) concentrations in the pellets were estimated by a thermal-optical-transmittance method (i.e., carbon analysis). From the EC concentration, the amount of ingested DEP or CB could be calculated. The described technique allowed the determination of the kinetics and dose dependence of DEP uptake by LA4 lung epithelial cells and MHS alveolar macrophages. Both cell types ingested DEP to a similar degree; however, the MHS macrophages took up significantly more CB than the epithelial cells. Cytochalasin D, an agent that blocks actin polymerization in the cells, inhibited approximately 80% of DEP uptake by both cell types, indicating that the process was actin-dependent in a manner similar to phagocytosis. This technique can be applied to examine the interactions between cells and particles containing EC and to study the modulation of particle uptake in diseased tissue.
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PMID:Isolation and quantitative estimation of diesel exhaust and carbon black particles ingested by lung epithelial cells and alveolar macrophages in vitro. 1847 33


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