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)

The arginine requirement of young, female rabbits was reassessed using body weight gain, serum arginine concentration and expired 14CO2 following administration of 14C arginine as criteria of adequacy. With a diet that provided the indispensable amino acids at the previously estimated requirement level the arginine requirement was estimated at or near 0.6% of diet by all three of the criteria studied. When an amino acid pattern based on isolated soy protein was fed, a significantly lower serum arginine level was observed with 0.6% dietary arginine corroborating that the arginine requirement may be higher when this pattern is fed than when the requirement pattern is fed. Liver arginase and kidney transamidinase levels were normal and were not influenced by dietary arginine levels over the range used. Serum creatine phosphokinase, which has been studied in other species in relation to muscular dystrophy, showed a significant increase with increasing dietary level of arginine. No signs of this disorder however, were observed. Adult male rabbits maintained body weight and showed no change in serum arginine concentration when both arginine and glycine were completely omitted from the diet.
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PMID:Further studies on the arginine requirement of the rabbit. 94 64

We have previously shown that diethylstilbestrol (DES) almost always, and prednisolone (Pr) less frequently, lowered the high serum enzyme activities in Duchenne's muscular dystrophy (DMD). In experimental studies, it was shown that pretreatment of mice with each of these agents lowered enzyme efflux from isolated skeletal muscle incubated in vitro, but efflux was augmented by higher doses of Pr. This suggested that these agents may influence skeletal muscle enzyme efflux in man also, producing the effects noted in DMD. The present studies were undertaken to assess the effect on enzyme efflux from skeletal muscle and heart that these two agents would exert when given in combination. Four groups of mice (14/group) were injected with saline, 250 mug DES, 35 mug Pr, or 250 mug DES plus 35 mug Pr in saline every other day for 22 days. The left gastroecnemius and heart were isolated from animals of each group, and placed in separate tubes containing incubation medium at 25 degrees C. The efflux of creatine phosphokinase (CPK) and lactate dehydrogenase (LDH) which issued from each organ was determined over a 5 hour period. In the doses tested, it was found that: 1) DES selectively reduced enzyme efflux from skeletal muscle, but had no effect on enzyme efflux from heart; 2) a Pr dose which decreased enzyme efflux from the heart, augmented efflux from the gastrocnemius; and 3) DES prevented the enhanced enzyme efflux produced by Pr. These studies indicate that these hormones, in pharmacological doses, influence enzyme efflux from muscle. This suggests, but it is not established, that these hormones also exert a similar physiological role. Finally, this experimental model appears to be useful in assessing the effects of single agents, and agents in combination, on enzyme efflux, and should be of aid in selecting appropriate agents which may be therapeutically useful in Duchenne's muscular dystrophy.
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PMID:Disparate effects of diethylstillbestrol and prednisolone on enzyme efflux from heart and skeletal muscle. 96 73

Determination of the creatine kinase isoenzyme pattern in 62 biopsy samples obtained from patients with neuromuscular disease revealed changes mainly in Duchenne muscular dystrophy. The BB isoenzyme was detected in 10 out of 17 cases with Duchenne muscular dystrophy and the relative amount of MB+BB isoenzyme was significantly increased in this group (P less than 0.005). In serum the MB isoenzyme was detected in all 28 cases with progressive muscular dystrophy and frequently also in other neuromuscular diseases. Among 152 control samples the MB isoenzyme was detected only in 2 cases. It is suggested that the finding of MB isoenzyme in the serum with normal or only slightly elevated total CK activity may be a further proof of neuromuscular disorder, but the finding is not specific for any particular disease.
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PMID:Creatine kinase isoenzymes in neuromuscular diseases. 97 5

The AA. have carried out in patients affected by proved myocardial infarct, by other cardiac diseases and by muscular dystrophy the following enzymes determinations: total CPK, total LDH, SGOT, SGPT, HBDH, CPK isoenzymes by column chromatography (Mercer's method) and LDH isoenzymes either by column chromatography (Mercer's method) and by electrophoretic separation. Some results concerning the appearance of the CK-MB isoenzymes during the acute period of the myocardial infarction are described.
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PMID:[Determination of CPK isoenzymes by column chromatography (author's transl)]. 102 94

Two experiments to study nutritional muscular dystrophy (N.M.D.) in calves were conducted in northern Ontario, where the disease is prevalent. In the first experiment, ninety Shorthorn cows were used. They were divided into three groups and fed the following forages during the winter of 1972-1973. Group I - Silage. Group II. - Heated-air-dried hay. Group III. - Field-dried hay. Chemical analysis of the forage during storage showed that the silage had a higher tocopherol content than the other two forages. This higher content had a direct effect on plasma tocopherol concentrations in the cows, since tocopherol levels were found to be higher in the group fed silage than in the other two groups. The highest mortality rate-eight dead calves-was in the group fed heated-air-dried hay; one calf died in each of the other two groups. Hence, it is evident that the severity of N.M.D. symptoms in calves is directly linked with the quantity of selenium or vitamin E ingested. The substances act synergistically to protect against the disease. In a second experiment, a herd of forty-seven Shorthorn cows, some of which had calves with N.N.D. and some of which had healthy offspring, were studied to measure the activity of serum creatine phosphokinase. The dams were found to have the same C.P.K., whether or not their calves suffered from N.M.D.
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PMID:[Vitamin E activity of stored forages and incidence of myopathy in calves]. 103 40

In 200 patients with neuromuscular diseases the author studied malonic dehydrogenase and lactic dehydrogenase activity comparing it with the activity of serum creatine kinase and aldolase. A significant rise in the values of all these enzymes was found only in the Duchenne type of muscular dystrophy, in polymyositis, and less frequently in the limb-girdle type of muscular dystrophy. Raised activity of creatine kinase and sidolase was observed in mothers and sisters of patients with Duchenne type of dystrophy, in patients with non-progressive myopathy, periodic paralysis, amyotrophic lateral sclerosis and polyneuropathy. With progression of dystrophy the activity of these enzymes decreases.
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PMID:[Serum enzymatic activity in neuromuscular diseases]. 112 44

Serum creatine phosphokinase activity increased significantly four or six hours after the administration of prednisolone in patients with muscular dystrophy of various types, whereas it did not increase in other muscular disorders. The extent of the response correlated inversely with the grade of disability. The prednisolone test may help to differentiate muscular dystrophy from polymyositis.
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PMID:Response of serum creatine phosphokinase to steroid hormone. 112 83

Problems of genetic counseling in connection with Duchenne progressi ve muscular dystrophy are discussed. The disorder is transmitted by a sex-linked recessive mechanism; thus, it rarely appears in women, although it is transmitted from mother to son. Sons of a carrier mother have a 50% chance of receiving the pathological chromosome (and manifesting the disorder clinically), while daughters have a 50% chance of being carriers themselves. Carriers may be identified from studies of family trees and by elevated creatine phosphokinase levels. In the case of a carrier who desires children, amniocentesis and termination of pregnancy in the case of a male fetus is the only possibility for preventing the birth of a boy with the disorder. Prospective parents must be informed of the risk as well as of the means of prevention.
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PMID:[Genetic counseling in progressive muscular dystrophy]. 112 31

We have previously shown that oral diethylstilbestrol (DES) lowers the high serum enzyme levels characteristic of Duchenne's muscular dystrophy (DMD). The present studies were undertaken to assess the effect of DES on the efflux of enzymes from isolated mouse skeletal muscle. Thirty-four male mice were used. Half received daily subcutaneous injections of 10 mug diethylstilbestrol-diphosphate (DES-DP) in saline for up to 3 wk and half daily saline injections. Left gastrocnemii were isolated from control and treated mice, and placed in separate incubation media at 37 degrees C. The efflux of creatine phosphokinase (CPK) and lactate dehydrogenase (LDH) from each was compared over a 4- to 5-hr period. In 15 of 18 there was a reduction in efflux of both enzymes from muscles isolated from DES-DP-treated mice. The greatest effect was noted during the second hour, when the mean efflux of each enzyme was reduced about 30%. Minor differences in muscle weight, water content, and enzyme activities did not explain the reduced efflux. These results suggest that DES has either reduced the permeability of the sarcolemma or enhanced the intracytoplasmic stability of these enzymes. This is the first drug reported to reduce the spontaneous enzyme efflux from isolated skeletal muscle. It remains to be established that a similar effect accounts for the reduction in serum enzyme levels when DES is administered to persons with DMD.
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PMID:Reduction of enzyme efflux from skeletal muscle by diethylstilbestrol. 114 55

Hereditary muscular dystrophy in chickens of the New Hampshire strain was treated with penicillamine from the 9th day after hatching to the 425th day. The adult maintenance dose for males was 50 mg/kg per day and for females, 13-65 mg/kg per day. In avian dystrophy, deterioration of the muscle fibers is evidenced in the 2nd mo by an inability of the birds to rise after falling on their backs and by a progressive rigidity of the wings. The drug delayed the onset of symptoms and partially alleviated the debilitating aspects of the disease. Penicillamine produced three major improvements: (a) better righting ability when birds were placed on their backs; (b) greater wing flexibility; (c) and suppression of plasma creatine phosphokinase activity. The results are statistically analyzed and discussed in relationship to Duchenne dystrophy. Normal birds were not affected by penicillamine as judged by these parameters. The rationale for using penicillamine, a sulfhydryl compound with reducing properties, was (a) to attempt to protect essential thiol enzymes in the anabolic and glycolytic pathways against inactivation and (b) to prevent collagen cross-linking and deposition in muscle. Although the precise mechanism of drug action has not been determined. the possible role of penicillamine in mitigating the symptoms of genetic dystrophy in man is under consideration. Further, penicillamine may have a more generalized application i the prevention of contractures in a variety of neuromuscular disorders.
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PMID:Beneficial effects of penicillamine treatment on hereditary avian muscular dystrophy. 115 90


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