Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A disorder of CNS myelination was found in paralytic tremor ("pt") rabbits. The condition is inherited in a sex-linked recessive mode. Ultrastructurally, an obvious myelin deficiency with aberration of myelin sheath formation is observed. The yield of myelin isolation was reduced to 20-30% of control. Myelin isolated from 4-week-old "pt" rabbits contained reduced amounts of galactosphingolipids and of several myelin protein markers. Moreover, myelin basic protein, analyzed by two-dimensional gel electrophoresis, showed a deficit in its more basic components. All these facts suggest a delay in myelin maturation. Ganglioside content was increased as well as Na+,K+-ATPase specific activity. 2',3'-Cyclic nucleotide phosphodiesterase (CNPase) specific activity was the same in "pt" as in control myelin but differed by having greater sensitivity to detergent activation.
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PMID:Myelin composition and activities of CNPase and Na+,K+-ATPase in hypomyelinated "pt" mutant rabbit. 282 82

Under gentle shaking, the phorbol 12,13-dibutyrate (P(Bu)2)-induced adhesion among human blood mononuclear leukocytes started within a few minutes, increased with time and was almost complete after 12 h. During this moment and thereafter more than 60% of the cells were in aggregates. Induction of the cell aggregation by 20 min treatment with P(Bu)2 did not occur in Ca++/Mg++-free medium but was almost complete with Mg++ alone and reached its maximal manifestation with both divalent cations present. The intracellular Ca++ antagonist 8-(N,N-diethylamino)octyl-3,4,5-trimethoxybenzoate had a minimal inhibitory effect, and simultaneous treatment of the cells with Ca++ ionophore A23187 and P(Bu)2 did not increase the intercellular binding, whereas A23187 alone induced some cell aggregation. Retinal, a protein kinase C inhibitor, inhibited the intercellular adhesion by more than 50%, and treatment of intact cells with 1-oleoyl-2-acetyl-glycerol, an activator of the enzyme, induced some cell aggregation that was slightly increased when A23187 was added simultaneously. Nordihydroguaiaretic acid, 5,8,11-eicosatriynoic acid and 5,8,11,14-eicosatetraynoic acid, which inhibit lipoxygenation of arachidonic acid, reduced the cell aggregation in contrast to indomethacin and acetylsalicylic acid that had no effect. Cellular ATPases, inhibited by quercetin, but not the ouabain-sensitive Na+, K+-ATPase, appeared to participate, whereas the amiloride-sensitive plasma membrane Na+/H+ exchanger and the intracellular levels of cGMP did not seem to influence the system.
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PMID:Phorbol ester-induced adhesion (binding) among human mononuclear leukocytes requires extracellular Mg++ and is sensitive to protein kinase C, lipoxygenase, and ATPase inhibitors. 293 88

Levels of ATPase activity were examined in the cells of Saccharomycopsis fibuligera during mycelial growth. The mycelial cells (M-form cells) grown under standing culture conditions exhibited a marked increase of ATPase activity after 3 days, showing a broad peak between 5 and 20 days and a maximum at 10 days. The dry weight/ml of the yeast-like cells (Y-form cells) grown under shaking culture conditions reached its peak after 3 days and remained constant throughout the 10 day period of observation. The dry weight/ml of the M-form cells under standing culture conditions exhibited its peak in 10-15 days and then gradually decreased. A very small amount of Y-form cells among the M-form cells under standing culture conditions, exhibited very low ATPase activity even in 10 days' incubation. In M-form cells, the particles increased in number during 20 days of incubation.
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PMID:ATPase activity in cells of the yeast Saccharomycopsis fibuligera during mycelial growth. 296 74

Activity of Na, K-ATPase in homogenates of fresh cerebral cortex of rats was compared with that of cortex frozen under different conditions. Activity yields after rapid in situ freezing of the exposed cerebral cortex were twice, higher (26.1 U) than in homogenates of the fresh cortex (13.3 U). Fresh brain kept on ice for 60 and 300 s and subsequently frozen in liquid nitrogen yielded activities comparable to those of the tissue frozen in situ (24.1 U and 24.9 U for 60 s and 300 s periods, respectively). Inhibition of Na, K-ATPase by 10(-7) M vanadate was significantly stronger (38%) in homogenates of the fresh brain then in those of the cortex frozen in situ (28%). High Na, K-ATPase activity (47.6 U) in suspensions of synaptosomal membranes (SM) prepared from fresh cortical homogenates was only slightly inhibited by 10(-7) M vanadate (12%). Various treatments of homogenates or SM suspensions, like increase of piston rotation speed, repeated freezing and thawing procedure or vigorous shaking did not significantly affect the enzyme activity. Mg-ATPase activity and its sensitivity to vanadate was also modified by tissue treatment but the effect was much less pronounced.
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PMID:What is the correct value for Na, K-ATPase activity in homogenates of cerebral cortex? 300 11

The Ca2+-ryanodine receptor complex is solubilized in functional form on treating sarcoplasmic reticulum (SR) vesicles from rabbit fast skeletal muscle with 3-[(3-cholamidopropyl)dimethylammonio]-1-propane-sulfonate (CHAPS) (1 mg/mg protein) and 1 M NaCl at pH 7.1 by shaking for 30 min at 5 degrees C. The heavy membrane preparations obtained from pyrophosphate homogenates frequently exhibit junctional feet and appear to be derived primarily from the terminal cisternae of the SR. The characteristics of [3H]ryanodine binding are similar for the soluble receptor and the heavy SR vesicles with respect to dependence on Ca2+, pharmacological specificity for inhibition by six ryanoids and ruthenium red, and lack of sensitivity to voltage-dependent Ca2+-channel blockers, inositol 1,4,5-trisphosphate, or doxorubicin. In contrast, the cation sensitivity is decreased on receptor solubilization. The soluble receptor is modulated by cyclic nucleotides and rapidly denatured at 50 degrees C. Saturation experiments reveal a single class of receptors (Kd = 9.6 nM), whereas kinetic measurements yield a calculated association constant of 5.5 X 10(6) min-1 M-1 and a dissociation constant of 5.7 X 10(-4) min-1, suggesting that the [3H]ryanodine receptor complex ages with time to a state which is recalcitrant to dissociation. Sepharose chromatography shows that the receptor complex consists primarily of two protein fractions, one of apparent Mr 150,000-300,000 and a second, the [3H]ryanodine binding component, of approximately Mr 1.2 X 10(6). Preliminary analysis of the soluble receptor preparation by sodium dodecyl sulfate-polyacrylamide gel electrophoresis reveals subunits of Mr greater than 200,000 and major bands of calsequestrin and Ca2+-transport ATPase. These findings indicate that [3H]ryanodine binds to the Ca2+-induced open state of the channel involved in the release of contractile Ca2+.
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PMID:Calcium-ryanodine receptor complex. Solubilization and partial characterization from skeletal muscle junctional sarcoplasmic reticulum vesicles. 372 65

Since we have demonstrated that ATPase system was sensitive to chlordecone, it was decided to examine the relationship between physiological and biochemical responses to this neurotoxin. Male Sprague-Dawley rats were fed with chlordecone by gastric intubation at 10, 25 and 50 mg/kg/day for three days. Control rats received 0.3 ml of corn oil. Complete body movements (including tremors) were monitored for a period of 12 hr at 24, 48 and 72 hr after treatment by a piezoelectric crystal attached to the bottom of a plastic rodent cage. The output of the crystal was recorded by a Grass model of EEG machine and magnetic tape. For biochemical study chlordecone treated rats were killed, the brain synaptosomes were prepared and Na+-K+ ATPase, oligomycin-sensitive and insensitive Mg2+ ATPases were determined. Rats receiving chlordecone showed an increased tremor activity which was significant and dose- dependent with a correlation coefficient of the regression line of 0.96. The onset of tremors was evident as early as 2 hr in 50 mg/kg dosed rats. Behavioral abnormalities include startling response to external stimuli like sound, etc. The brain synaptosomal Na+-K+ and oligomycin-sensitive Mg2+ ATPases were significantly decreased in chlordecone treated rats as compared to controls and the decrease was dose-dependent. A linear relationship was observed between the decreases in ATPase activities and physiological (tremor) activity with an r value of 0.96. These results suggest that the inhibition of ATPase system in brain may be related to the production of the neurotoxic symptoms.
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PMID:Acute chlordecone toxicity in rats: a relationship between tremor and ATPase activities. 617 57

We generated mice, null mutant in the adhesion molecule on glia (AMOG), the beta 2 subunit of the murine Na,K-ATPase gene. These mice exhibit motor incoordination at 15 d of age, subsequently tremor and paralysis of extremities, and die at 17-18 d after birth. At these ages, the mutants have enlarged ventricles, degenerating photoreceptor cells, and swelling and degeneration of astrocytic endfeet, leading to vacuoles adjoining capillaries of brain stem, thalamus, striatum, and spinal cord. In tissue homogenates from entire brains of 16-17-d-old mutants, Na,K-ATPase activity and expression of the beta 1 subunit of the Na,K-ATPase and of the neural adhesion molecules L1, N-CAM, and MAG appear normal. We suggest that the mutant phenotype can be related primarily to reduced pump activity, with neural degeneration as a possible consequence of osmotic imbalance.
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PMID:Degeneration of neural cells in the central nervous system of mice deficient in the gene for the adhesion molecule on Glia, the beta 2 subunit of murine Na,K-ATPase. 752 97

The adhesion molecule on glia (AMOG) has been reported to function as cell adhesion molecule and also to constitute the beta 2-subunit of the murine Na,K-ATPase. In order to elucidate these functions in vivo, Magyar et al. have generated mice carrying a targeted deletion of the AMOG gene. These mice exhibit behaviourally normal development till postnatal day P16. At this time, they develop muscular weakness, incoordination, and tremor. Death invariably occurs 24-36 hours after onset of the symptoms. Histological and ultrastructural examination of brain sections show enlarged ventricles, brain edema, and swelling of astrocyte end feet. However, no disturbances of the architecture or cell migration in the brain can be detected. In order to identify long-term consequences of AMOG deficiency which might not yet be detectable at the time of death, we have established a CNS grafting model. The embryonal brain anlage (E10.5-E13.5) was grafted into the caudoputamen of wild type mice. The graft recipients are sacrificed up to 7 months after the procedure. Both wild type and AMOG deficient grafts develop and form solid neural tissue with neurons, myelinated axons, glial cells, and ventricular structures, as shown by histological and immunocytochemical analysis. However, no differences in grafts derived from wild type, heterozygous, and AMOG-deficient donors can be detected. Proliferation has been examined by BrdU immunocytochemistry. The blood-brain barrier as examined by repeated magnetic resonance imaging after injection of Gadolinium-DTPA has been shown to be largely reconstituted five weeks after grafting.
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PMID:[Morphology and development of neural transplants of AMOG-deficient mice]. 753 17

Adhesion molecule on glia (AMOG) represents the beta 2-subunit of murine Na,K-ATPase. Mice carrying a targeted deletion of the AMOG/beta 2 gene exhibit tremor and limb paralysis at postnatal day (P) 15 and die 2 days after the onset of symptoms. The brains of these mice show edema and swelling of astrocytic end feet. However, the cause of death has remained unclear. To identify long-term consequences of AMOG/beta 2 deficiency, we have grafted parts of the embryonic telencephalic anlage of AMOG/beta 2-deficient mice into the caudoputamen of wild-type mice and analyzed the grafts up to 500 days after transplantation. Histological, immunocytochemical, and in situ hybridization techniques were applied to examine histoarchitecture, proliferation, differentiation, and long-term survival of grafts. AMOG/beta 2-deficient telencephalic grafts develop normally and form solid neural tissue that cannot be distinguished from control grafts by morphological features or with immunocytochemical stains for neuronal and glial markers. No signs of degeneration can be found. Expression analysis, however, revealed that no AMOG/beta 2 protein of possible host origin can be detected in AMOG/beta 2-deficient grafts. Graft-borne astrocytes express neither the AMOG/beta 1 nor the AMOG/beta 2 subunit of Na,K-ATPase as examined with immunocytochemistry and in situ hybridization. These findings indicate that AMOG/beta 2 is not necessary for long-term survival of telencephalic graft tissue.
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PMID:The AMOG/beta 2 subunit of Na,K-ATPase is not necessary for long-term survival of telencephalic grafts. 892 33

Scrapie, one of the prion diseases, is a transmissible neurodegenerative disease of sheep and other animals. Clinical symptoms of prion diseases are characterized by a long latent period, followed by progressive ataxia, tremor, and death. To study the induction of neurodegeneration during scrapie infection, we have analyzed the activities of various antioxidant enzymes and mitochondrial enzymes in cerebral cortex, brain stem, and cerebellum of scrapie-infected hamsters. The activity of mitochondrial Mn-superoxide dismutase (SOD) was decreased, while the activities of cytosolic Cu/Zn-SOD and catalase were not altered in infected brains. The activities of glutathione peroxidase and glutathione reductase were increased in scrapie-infected hamsters. The decreased activity of Mn-SOD might result in increasing oxidative stress in the mitochondria of infected brain; this concept is supported by our findings of a high level of lipid peroxidation, and low levels of ATPase and cytochrome c oxidase activity in the infected cerebral mitochondria. In addition, structural abnormalities of mitochondria have been observed in the neurons of hippocampus and cerebral cortex of infected brain. These results suggest that mitochondrial dysfunction caused by oxidative stress gives rise to neurodegeneration in prion disease.
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PMID:Mitochondrial dysfunction induced by oxidative stress in the brains of hamsters infected with the 263 K scrapie agent. 975 61


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