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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-eight Holstein heifer calves were allotted at birth to one of four treatments: 1) 0 mg, 2) 1,400 mg, or 3) 2,800 mg of dl-alpha-tocopherol acetate given orally at weekly intervals, or 4) 1,400 IU of dl-alpha-tocopherol weekly by intramuscular injection in order for us to study their performance and metabolic profile. Calves were fed milk at 8% of birth weight until they were weaned at 6 wk of age and fed a complete calf starter ad libitum from birth. Calves were on experiment for 12 wk. There were no significant differences in weekly weight gains, starter consumption, and fecal scores among treatments. However, there was a trend toward greater starter consumption and weight gains in supplemental calves. Serum alpha-tocopherol concentration measured after 7 d of each administration was significantly higher at wk 4 in calves given the high oral supplementation and at wk 2, 4, 6, and 8 higher in injected calves than in unsupplemented calves. Creatine kinase activity was higher in unsupplemented calves and negatively correlated with serum alpha-tocopherol until wk 8, suggesting preclinical muscular dystrophy. Alkaline phosphatase activity was higher with the high oral supplementation. Serum carbon dioxide values showed a trend toward positive correlation with those for serum tocopherol; however, the values were within normal range. There were no significant differences in creatinine, glucose, phosphorus, calcium, urea nitrogen, chloride, sodium, potassium, albumin, and total protein among treatments. Serum glucose was higher in all calves at wk 10 and 12 than at wk 4, 6, and 8. Calves may not get enough vitamin E with conventional calf starters, and supplementation may be essential to obtain maximum performance.
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PMID:Effects of supplemental vitamin E on the performance and metabolic profiles of dairy calves. 406 45

Because the erythrocyte (RBC) in Duchenne's muscular dystrophy (DMD) is thought to be a suitable experimental paradigm for the sarcolemma, the RBC membrane-bound enzyme (Ca2+ + Mg2+)-ATPase has been investigated as to its relevance to abnormalities of calcium metabolism in DMD muscle. In this study, RBC (Ca2+ + Mg2+)-ATPase activity, intracellular calcium and potassium contents and complete hemogram were examined in 10 DMD patients and 16 age-matched controls. (Ca2+ + Mg2+)-ATPase activity was found elevated in the DMD RBC, consistent with reports from previous studies, but no abnormalities in intracellular calcium, potassium or hemograms were detected. It seems that although the (Ca2+ + Mg2+)-ATPase activity is changed, it bears no relevance to calcium homeostasis in DMD RBC. It is inferred that the increase in intramuscular calcium in DMD muscle, which is also found in other neuromuscular diseases, may be a non-specific finding in the diseased muscle and part of the final common pathway leading toward cellular degeneration and death.
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PMID:Erythrocyte (Ca2+ + Mg2+)-ATPase activity and calcium homeostasis in Duchenne muscular dystrophy. 614 34

Potassium and sodium ion-selective microelectrodes were used in vitro to investigate the depolarization of skeletal muscle fibers associated with muscular dystrophy. In dystrophy there was a large increase of intracellular Na activity and an associated decrease in K activity in fibers of extensor digitorum longus muscles. Despite this, the recorded membrane potential was very close to the calculated potassium equilibrium potential (Ek) in dystrophic fibers. In contrast, in normal muscle fibers, Em was significantly depolarized with respect to Ek. The data suggest that in dystrophic fibers there is an increase in the relative membrane permeability to potassium over sodium.
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PMID:Intracellular activity of sodium in normal and dystrophic skeletal muscle from C57BL/6J mice. 669 62

Many children with muscular dystrophy are overweight, and although weight control is pursued in some centres it is unusual to encourage severe dietary restriction for fear that it might lead to accelerated loss of muscle. In this study, two overweight boys with muscular dystrophy were monitored by whole-body nitrogen balance, total body potassium, strength and functional measurements during calorie restriction. Both patients were found to have a transient loss of nitrogen on commencing the low calorie intake: thereafter, weight loss was not found to have any deleterious effect on muscle bulk or function in either patient. It is suggested that controlled weight-reduction in obese children with muscular dystrophy is a safe and practical way of losing excess fat, which can improve mobility and self-esteem, and may possibly effect longevity.
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PMID:Weight reduction in boys with muscular dystrophy. 673 55

Myotonic muscular dystrophy (MyD) is an inherited human disease involving skeletal muscle as well as many other organ systems. We have approached the study of this disorder by growing normal and diseased human muscle in a primary tissue culture system and investigating some of the electrical properties of the resulting myotubes. The most distinctive abnormality noted in MyD myotubes was an increased tendency to fire repetitive action potentials. A decreased action potential afterhyperpolarization amplitude and the presence of depolarizing afterpotentials were also noted, as were a decreased resting membrane potential, decreased action potential amplitude and overshoot, and decreased outward-going rectification. Although the ionic basis of these abnormal properties in vitro is not clearly defined, changes in the slow outward-going potassium current offer the best explanation. Furthermore, MyD cell culture offers a valuable model for critical analysis of the molecular mechanisms underlying MyD deficits.
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PMID:Cultured muscle from myotonic muscular dystrophy patients: altered membrane electrical properties. 694 Dec 62

At least three myopathies have been associated with malignant hyperthemia (MH). The clinical manifestations of MH are variable and depend on the nature of the underlying myopathy and the anesthetic agents administered. Unless muscle relaxants are used, fever and muscle rigidity may be delayed at onset. Tachycardia and tachypnea are often the earliest manifestations and can occur immediately or several hours into a surgical procedure. Life-threatening cardiac arrhythmias may result from hyperkalemia and acidosis. A hyperthermic reaction developed in an 8-year-old boy with a family history of Duchenne's muscular dystrophy one hour after induction of anesthesia. Temperature elevation and muscle rigidity were minor components of the condition. Determination of arterial blood gas concentrations and the serum potassium level established the diagnosis and enabled the start of lifesaving therapy.
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PMID:Malignant hyperthermia. Current concepts. 706 79

Myotonic muscular dystrophy (MyD) is a systemic genetic disorder that is thought to result from a generalized cellular membrane defect although the exact nature of this defect is unknown. This study examines two calcium-dependent membrane processes that have been observed in erythrocytes from healthy individuals: calcium-stimulated phosphatidic acid accumulation and calcium-induced potassium leak. We find that erythrocytes from MyD patients, in contrast to controls, have markedly impaired phosphatidic acid accumulations while maintaining normal potassium leaks. The calcium uptakes and ATP contents of MyD erythrocytes are not different from controls. We conclude that phospholipid metabolism is altered in MyD erythrocytes. The specificity of this abnormality and its relationship to altered muscular function are not known.
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PMID:Myotonic muscular dystrophy: defective phospholipid metabolism in the erythrocyte plasma membrane. 741 May 51

Three experiments were carried out with male broiler chickens reared from day- old to 6 weeks of age on semi-purified diets containing 10% fresh (Expt. 1 and 3) or oxidized (Expt. 2) re-esterified triglycerides with a fatty acid composition similar to that of soya bean oil containing increasing concentrations of either a mixture of d-alpha-, gamma-, delta-tocopherylacetate (d-tocopherols) of natural source or dl-alpha- tocopheryl acetate (dl-tocopherol). In Expt. 1 and 2 the mixture of d-tocopherols consisted of 35.7% d-alpha-, 45.3% d-gamma- and 19.0% d-delta-, while in Expt. 3 the distribution was 25.3% d-alpha-, 28.1% d-gamma- and 10.8% d-gamma- in 35.8% re-esterified triglycerides. The relative biopotency of d-alpha-: gamma-: delta-tocopherol was anticipated to be 100:25:1, whereas that of dl-alpha-tocopherol was 74% relative to d-alpha-tocopherol. The experiments demonstrate that the results obtained for the biological activity depend on the response parameters chosen. With respect to gain in weight, feed conversion, relative organ weight, packed cell volume (PCV), ELP (erythrocyte lipid peroxidation), plasma activities of glutamate-oxaloacetate-transaminase (GOT), creatine kinase (CK) and glutathione peroxidase (GSH-Px) and plasma Na+ concentration, the mixture of natural source tocopherols was identical to that of dl-alpha-tocopheryl acetate, although the concentration of alpha-tocopherol was only about one third of that of dl-alpha-tocopherol. Differences between natural source and synthetic tocopherols were expectedly observed with respect to plasma concentrations of alpha-, gamma-, delta-tocopherol. Differences between the two forms as to muscular dystrophy, in vitro haemolysis and potassium concentration in plasma were ambiguous. It is suggested that the function of d-alpha-, gamma-, delta-tocopherol in erythrocyte fragility and skeletal muscle structure should be compared to that of dl-alpha-tocopherol in future investigations.
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PMID:The biological activity of natural source tocopherols in chickens fed fresh or oxidized fat rich in linoleic acid. 821 3

We report two boys aged 4 and 10 months who suffered cardiac arrests after induction of anaesthesia. Both infants had no personal or family history of myopathy. In both cases anaesthesia was induced by inhalation with halothane and N2O/O2 (70/30). To facilitate tracheal intubation both were given succinylcholine after the administration of atropine. The 4-month-old developed muscle rigidity and cardiac arrest occurred immediately after tracheal intubation. Resuscitation was unsuccessful. Laboratory findings during resuscitation showed elevated serum potassium levels of more than 10 mmol/l and serum creatine phosphokinase 17.700 IU/l. Histopathologic examination of the skeletal muscle revealed congenital muscular dystrophy. In the older boy no muscle contractures were noted after administration of succinylcholine. He developed bradycardia that progressed to asystole 15 min after induction of anaesthesia. After 1 h of resuscitation a sinus rhythm could be established. The boy developed myoglobinuria and his serum creatine phosphokinase reached a maximum level of 45,000 IU/l on the 2nd day. The child survived and made a complete recovery. Two months later a muscle biopsy taken from the quadriceps showed marked muscular dystrophy. Duchenne's muscular dystrophy could be excluded. The most likely underlying reasons for these complications are discussed: anaesthesia-induced acute rhabdomyolysis or malignant hyperthermia.
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PMID:[Anesthetic-induced heart arrest. A case report of 2 infants with previously unrecognized muscular dystrophy]. 844 72

Hemolytic anemia developed in a male who had been diagnosed as having myotonic muscular dystrophy (MMD). His red cell life-span examined by 51Cr-labeling method was shortened (T 1/2 = 6.5 days). Specific abnormalities of red cells were not found other than increased osmotic resistance, increased intracellular sodium, and decreased intracellular potassium of red cells. A clinical review of 18 other patients with MMD did not reveal any signs of hemolysis. It may be suggested that the underlying red cell membrane defects due to MMD contributed to this rare association with hemolysis.
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PMID:Hemolytic anemia associated with myotonic muscular dystrophy. 891 5


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