Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intraruminal selenium soluble-glass boluses were administered by balling gun to 65 of 125 crossbred beef cows (Shorthorn X Charolais) during the last trimester of pregnancy. Elevated (P less than .01) whole blood glutathione peroxidase (GSH-Px) concentrations were observed monthly for the next 10 mo following initiation of treatment, reaching the maximum magnitude (263 vs 41) at the fourth month. Monthly milk samples showed elevated selenium concentrations (P less than .01, April through August; P less than .05 through September). Intraruminal, selenium soluble-glass bolus administration to gestating cows was highly effective in raising the selenium status of their progeny. Although the control calves were in low-selenium status, no acute cases of nutritional muscular dystrophy were observed during this experiment.
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PMID:Effect of intraruminally administered, selenium soluble-glass boluses on selenium status in cows and their calves. 366 43

Studies in experimental muscular dystrophy indicate a possible role for anomalous redox metabolism in the genesis of these disorders, prompting a retrospective review of changes in redox-active enzymes in Duchenne muscular dystrophy (DMD). Both manganous and copper-zinc superoxide dismutase (Mn and CuZn SOD) content and glutathione peroxidase and catalase activities were measured in muscle biopsy specimens taken from normal individuals and from patients with Duchenne muscular dystrophy and other neuromuscular diseases. Muscle from patients with Duchenne dystrophy differed from the norm in that both Mn SOD and CuZn SOD were decreased and glutathione peroxidase was increased. This profile differed from that in anterior horn cell diseases in that CuZn SOD was not decreased in these disorders and from polymyositis, where CuZn SOD was decreased without an increase in glutathione peroxidase. Thus, there appears to be disease-specific changes in these enzymes in DMD. These data support the concept that changes in redox-active enzymes may be associated with the genesis of DMD.
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PMID:Superoxide dismutases, glutathione peroxidase, and catalase in neuromuscular disease. 382 87

An inherited form of muscular dystrophy in chickens has been used as a model of Duchenne muscular dystrophy. The pectoralis major muscle of chickens with this disease showed a significantly elevated activity of catalase (CAT) one day after hatching, and by 7 days showed elevated superoxide dismutase (SOD), glutathione peroxidase (GPX) and glutathione-S-transferase (GST) activities. Increases were also found in tissues of the dystrophic birds that, unlike the pectoralis muscle, are considered to be unaffected by the pathology of muscular dystrophy. The soleus muscle contained significantly increased levels of SOD and GPX in 1 and 7 day old chickens, and increased GST in 1, 14, and 28 day old birds. CAT was significantly increased in liver from 1 and 7 day old chickens, while GPX was increased in lung from 1, 7 and 14 day old birds. These results support the possibility that excess oxygen free-radicals or altered cellular antioxidant defenses play some role in the pathogenesis of muscular dystrophy.
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PMID:Activities of antioxidant enzymes in muscle, liver and lung of chickens with inherited muscular dystrophy. 394 39

Administration of an intraruminal selenium pellet to a herd of pregnant crossbred cows was evaluated for controlling nutritional muscular dystrophy in an area of northern Ontario with numerous losses of calves. Cows were winter-fed grass silage. Each spring cows and calves went to pasture. A single dose of intraruminal selenium pellet was given to 80 cows during last 3 mo of pregnancy the 1st yr only while the remaining 80 were controls. During 3 consecutive years, efficacy of intraruminal selenium pellet was evaluated by selenium status of recipient cows and their offspring as well by the incidence of nutritional muscular dystrophy. Selenium in plasma, as well as glutathione peroxidase in whole blood, in the cows administered intraruminal selenium pellet, were higher than in the deficient controls. Ten months after intraruminal selenium pellet treatment, selenium in tissues was higher in treated than in untreated cows but within normal ranges. Before cows were turned out to pasture the 1st yr, milk selenium of intraruminal selenium pellet cows were higher than controls. This technique of selenium dosing was effective in raising the selenium status of the progeny. There was no evidence of nutritional muscular dystrophy in calves from selenium-dosed cows, while 15 calves born of the untreated cows showed clinical symptoms of nutritional muscular dystrophy.
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PMID:Intraruminal selenium pellet for control of nutritional muscular dystrophy in cattle. 398 Aug 11

Nine children with Duchenne muscular dystrophy were given Sanorex (mazindol), a growth hormone inhibitor, daily for 6 months. There was no significant change in their muscle function, but there was a significant reduction in weight gain and in levels of growth hormone, somatomedin C, hair zinc, serum zinc, and serum LDH. Selenium and glutathione peroxidase in the serum increased significantly. Thirteen other children with growth hormone deficiency had a significant reduction in hair selenium following growth hormone administration. These results show a significant relationship between growth hormone and selenium nutritional status and confirm our previous reports indicating an effect of growth hormone on zinc nutritional status. It is possible that prolonged therapy with a growth hormone inhibitor would attenuate the course and improve the longevity of patients with muscular dystrophy.
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PMID:Growth hormone inhibition causes increased selenium levels in Duchenne muscular dystrophy: a possible new approach to therapy. 649 89

Lipid peroxidation and other free radical reactions are known to disrupt and damage cellular structures and function, and it has been postulated as possible mechanisms of cellular damage of muscular dystrophy because increased levels of thiobarbituric acid (TBA)-reactive products and increased activities of superoxide dismutase and glutathione peroxidase were reported in avian muscular dystrophy. We reported that activities of superoxide dismutase, catalase, glutathione peroxidase and glutathione reductase were significantly increased in avian muscular dystrophy from the early developmental stage. Since these enzymes protect cellular structures from free radicals and peroxides, increased activities of these enzymes would indicate increased formation of radicals. Then it seems to be important to assay TBA-reactive products which indicate tissue malondialdehyde content, a by-product of lipid peroxidation. We used dystrophic chickens of New Hampshire series line 413 and their controls line 412 for assay of TBA-reactive products. Four or five birds from respective lines were killed by decapitation two weeks, four weeks and four months after hatching. The superficial pectoral muscle was immediately weighed and levels of TBA-reactive products in the muscle homogenate was assayed by fluorophotometry according to the modified method of Ohkawa and Tanizawa. Levels of TBA-reactive products were significantly higher in dystrophic chickens at all stages of development studied than those of the control group. At two weeks of age morphological changes are minimum if present and increased levels of TBA-reactive products cannot be considered as a secondary change of morphological alterations. Therefore, the results indicate involvement of lipid peroxidation damage in pathogenesis of this avian muscular dystrophy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Studies on pathogenesis of muscular dystrophy: levels of thiobarbituric acid-reactive products in avian muscular dystrophy]. 674 4

When blood selenium concentrations and glutathione peroxidase activity was measured in 30 standardbred horses a significant correlation was found (r = 0.97). A comparison between blood GSH-px activity in clinically healthy foals, foals affected by muscular dystrophy (MD) and their respective mares was also done. There was no difference in GSH-px activity between the healthy foals and the MD foals or between the corresponding mares.
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PMID:Glutathione peroxidase and selenium in the blood of healthy horses and foals affected by muscular dystrophy. 717 98

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

The concentration of selenium (Se) and the activity of glutathione peroxidase (GSH-Px) in plasma and erythrocytes were measured in healthy men and in patients with Duchenne-type progressive muscular dystrophy (DMD). In healthy men, the Se concentration in erythrocytes showed a steep rise with aging and ascended gradually in plasma. The GSH-Px activity in both plasma and erythrocytes clearly increased with aging. The relationship between the Se concentration and the GSH-Px activity in healthy men showed a parallel rise with aging, but the coefficients of correlation were not very high (r = 0.44 and 0.56 in plasma and erythrocytes, respectively. In DMD patients, on the other hand, the Se concentration in erythrocytes decreased steeply with aging, and it decreased gradually in plasma. The GSH-Px activity in both plasma and erythrocytes apparently increased as in healthy men with aging, but the level was about 80% of that of healthy men. When selenite (Se+4) is added to the whole blood in vitro at 25 degrees C, it is rapidly taken up by erythrocytes (within several minutes) and is then released into plasma (a period of 30 min), then subsequent reuptake by erythrocytes is proceeded slowly. Our attention was attracted to the pattern of selenite release from erythrocytes of DMD patients.
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PMID:[Selenium concentration in blood and Duchenne-type progressive muscular dystrophy]. 858 80

The mechanisms primarily responsible for the degenerative processes occurring in dystrophic skeletal muscle remain unresolved. The identification of the mechanisms that lead to the complete sparing of extraocular muscle in dystrophinopathies is of particular interest. A number of studies have provided evidence to suggest that the muscle pathology that characterizes muscular dystrophy may be, in part, free radical mediated. In the present study, we examined the antioxidant enzyme status of extraocular, diaphragm and gastrocnemius muscles in control strain and mdx mice. Our results revealed that in the control strain, both extraocular and diaphragm muscles had higher copper/zinc superoxide dismutase, manganese superoxide dismutase and selenium dependent glutathione peroxidase activities as compared to the gastrocnemius. Furthermore, the diaphragm had higher glutathione reductase activity as compared to the gastrocnemius. These findings indicate that the highly aerobic extraocular and diaphragm muscles have higher antioxidant enzyme capacity than the gastrocnemius, a muscle more dependent on anaerobic energy metabolism. Changes in the antioxidant enzyme status of the mdx mouse correlated, in part, with the degree of histopathological involvement of the three muscle groups assessed.
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PMID:Extraocular, limb and diaphragm muscle group-specific antioxidant enzyme activity patterns in control and mdx mice. 885 50


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