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Selenium in health and disease: a review.
Foster LH; Sumar S
Nutrition Research Centre, South Bank University, London,
UK.
Crit Rev Food Sci Nutr (United States) Apr 1997, 37 (3)
p211-28
Selenium (Se) was discovered 180 years ago. The
toxicological properties of Se in livestock were recognized
first; its essential nutritional role for animals was
discovered in the 1950s and for humans in 1973. Only one
reductive metabolic pathway of Se is well characterized in
biological systems, although several naturally occurring
inorganic and organic forms of the element exist. The amount
of Se available for assimilation by the tissues is dependent
on the form and concentration of the element. Se is
incorporated into a number of functionally active
selenoproteins, including the enzyme glutathione peroxidase,
which acts as a cellular protector against free radical
oxidative damage and type 1 iodothyronine 5'-deiodinase which
interacts with iodine to prevent abnormal hormone metabolism.
Se deficiency has been linked with numerous diseases,
including endemic cardiomyopathy in Se-deficient regions of
China; cancer, muscular dystrophy, malaria, and cardiovascular
disease have also been implicated, but evidence for the
association is often tenuous. Information on Se levels in
foods and dietary intake is limited, and an average
requirement for Se in the U.K. has no been established.
Available data suggest that intake in the U.K. is adequate for
all, except for a few risk groups such as patients on total
parenteral nutrition or restrictive diets. (122 Refs.)
Assessment of selenium and vitamin E deficiencies
in dairy herds and clinical disease in calves.
Zust J; Hrovatin B; Simundic B
Institute for Hygiene and Pathology of Animal Nutrition,
Veterinary Faculty Ljubljana, Slovenia.
Vet Rec (England) Oct 19 1996, 139 (16) p391-4
Because of the very low concentrations of selenium in the
dry matter of grass, grass silage, hay and maize silage
Slovenian dairy herds need to be supplemented with selenium.
Selenium in the form of mineral and feed mixtures maintained
adequate mean (sd) blood serum selenium concentrations of 43.9
(27.6) to 65.3 (18.5) micrograms/litre in lactating cows, but
in late lactation and in the dry period when only mineral
mixtures were used, about 60 per cent of the cows had marginal
serum selenium concentrations, mainly because of the low
intake of the mineral supplement. In 18 herds which were
either unsupplemented or irregularly supplemented with
selenium, the mean (sd) concentrations in blood serum were
13.7 (5.5) micrograms/litre and 17.4 (9.2) micrograms/litre,
respectively, for selenium and 2.98 (2.72) mg/litre and 1.62
(1.73) mg/litre for vitamin E, indicating that under extensive
farming conditions in Slovenia the lack of both micronutrients
may be responsible for nutritional muscular dystrophy
in calves. Among 37 clinical cases, cardiorespiratory signs
predominated in 25 of the calves and skeletal myopathy was
dominant in 12. A very low mean serum selenium concentration
[9.7 (7.2) micrograms/litre] and typically high activities of
aspartate aminotransferase (AST) [1125 (373) U/litre] and
creatine kinase (CK) [9169 (3681) U/litre) were observed for
the myocardial form of the disease, and 2797 (550) U/litre and
22,650 (13,500) U/litre were observed for the skeletal form of
the disease. A highly significant (P < 0.0001) difference
in the selenium concentration of liver dry matter between the
regularly supplemented [402 (207) micrograms/kg] and
irregularly supplemented [173 (69) micrograms/kg] herds was
observed. If a minimum value of 300 micrograms/kg of liver dry
matter is accepted as the criterion for the determination of
adequate selenium status, 93 per cent of the samples from the
irregularly supplemented herds were selenium deficient. A
similar proportion was estimated to be selenium deficient when
the criterion was taken to be 30 micrograms selenium/litre of
blood serum.
Wheat kernel ingestion protects from progression of
muscle weakness in mdx mice, an animal model of Duchenne
muscular dystrophy.
Hubner C; Lehr HA; Bodlaj R; Finckh B; Oexle K; Marklund SL;
Freudenberg K; Kontush A; Speer A; Terwolbeck K; Voit T;
Kohlschutter A
Department of Neuropediatrics, Virchow Medical Center,
Humboldt University, Berlin, Germany.
Pediatr Res (United States) Sep 1996, 40 (3)
p444-9
A simple, reproducible test was used to quantify muscle
weakness in mdx mice, an animal model of Duchenne muscular
dystrophy. The effect of bedding on wheat kernels and of
dietary supplementation of alpha-tocopherol on the progression
of muscle weakness was investigated in mdx mice. When measured
during the first 200 d of life, mdx mice developed muscle
weakness, irrespective of bedding and diet. When kept on wood
shavings and fed a conventional rodent diet, mdx mice showed
progressive muscle weakness over the consecutive 200 d, and
eventually showed a significant weight loss during the next
200-d observation period. Progression of muscle weakness and
weight loss were almost completely prevented in mdx mice that
were kept on wheat kernel bedding. In contrast, only
incomplete maintenance of muscle strength and body weight was
observed in mdx mice kept on wood shavings and fed the
alpha-tocopherol-supplemented diet. It is concluded from these
experiments that a component of wheat kernels other than
alpha-tocopherol is essential to prevent the progression of
muscle weakness in mdx mice.
Extraocular, limb and diaphragm muscle
group-specific antioxidant enzyme activity patterns in control
and mdx mice.
Ragusa RJ; Chow CK; St. Clair DK; Porter JD
Department of Anatomy and Neurobiology, University of
Kentucky Medical Center 40536-0084, USA.
J Neurol Sci (Netherlands) Aug 1996, 139 (2)
p180-6
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.
Aortic and iliac artery thrombosis in calves: nine
cases (1974-1993).
Morley PS; Allen AL; Woolums AR
Department of Veterinary Internal Medicine, Western College
of Veterinary Medicine, University of Saskatchewan, Saskatoon,
Canada.
J Am Vet Med Assoc (United States) Jul 1 1996, 209 (1)
p130-6
OBJECTIVE--To identify common clinical and diagnostic
features of calves with aortic or iliac artery thrombosis that
might aid in antemortem diagnosis of this condition.
DESIGN--Retrospective case series.
ANIMALS--9 calves < or = 6 months old in which aortic or
iliac artery thrombosis was confirmed at necropsy.
RESULTS--All calves had an acute onset of paresis or
flaccid paralysis of 1 or both hind limbs. Affected limbs were
hypothermic and had diminished spinal reflexes and diminished
pulse pressures. Diagnosis was definitively established in 2
calves by use of angiography. All 9 calves died or were
euthanatized.
CLINICAL IMPLICATIONS--This condition is rare and could be
mistaken for more common diseases of young cattle, such as
traumatic injury of the axial or appendicular skeleton,
vertebral osteomyelitis, nutritional muscular dystrophy
associated with vitamin E or selenium deficiency, injury to
the sciatic or femoral nerves, or clostridial myositis.
[Selenium concentration in blood and Duchenne-type
progressive muscular dystrophy]
Yamaguchi T
Department of Hygiene, Miyazaki Medical College.
Nippon Rinsho (Japan) Jan 1996, 54 (1) p134-40
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.
Two successful double-blind trials with coenzyme
Q10 (vitamin Q10) on muscular dystrophies and neurogenic
atrophies.
Folkers K, Simonsen R
Institute for Biomedical Research, University of Texas at
Austin 78705, USA.
Biochim Biophys Acta 1995 May 24;1271(1):281-6
Coenzyme Q10 (vitamin Q10) is biosynthesized in the human
body and is functional in bioenergetics, anti-oxidation
reactions, and in growth control, etc. It is indispensable to
health and survival. The first double-blind trial was with
twelve patients, ranging from 7-69 years of age, having
diseases including the Duchenne, Becker, and the limb-girdle
dystrophies, myotonic dystrophy. Charcot-Marie-Tooth disease,
and the Welander disease. The control coenzyme Q10 (CoQ10)
blood level was low and ranged from 0.5-0.84 microgram/ml.
They were treated for three months with 100 mg daily of CoQ10
and a matching placebo. The second double-blind trial was
similar with fifteen patients having the same categories of
disease. Since cardiac disease is established to be associated
with these muscle diseases, cardiac function was blindly
monitored, and not one mistake was made in assigning CoQ10 and
placebo to the patients in both trials. Definitely improved
physical performance was recorded. In retrospect, a dosage of
100 mg was too low although effective and safe. Patients
suffering from these muscle dystrophies and the like, should
be treated with vitamin Q10 indefinitely.
Biochemical rationale and the cardiac response of
patients with muscle disease to therapy with coenzyme
Q10.
Folkers K, Wolaniuk J, Simonsen R, Morishita M, Vadhanavikit
S
Proc Natl Acad Sci U S A 1985 Jul;82(13):4513-6
Cardiac disease is commonly associated with virtually every
form of muscular dystrophy and myopathy. A double-blind and
open crossover trial on the oral administration of coenzyme
Q10 (CoQ10) to 12 patients with progressive muscular
dystrophies and neurogenic atrophies was conducted. These
diseases included the Duchenne, Becker, and limb-girdle
dystrophies, myotonic dystrophy, Charcot-Marie-Tooth disease,
and Welander disease. The impaired cardiac function was
noninvasively and extensively monitored by impedance
cardiography. Solely by significant change or no change in
stroke volume and cardiac output, all 8 patients on blind
CoQ10 and all 4 on blind placebo were correctly assigned (P
less than 0.003). After the limited 3-month trial, improved
physical well-being was observed for 4/8 treated patients and
for 0/4 placebo patients; of the latter, 3/4 improved on
CoQ10; 2/8 patients resigned before crossover; 5/6 on CoQ10 in
crossover maintained improved cardiac function; 1/6 crossed
over from CoQ10 to placebo relapsed. The rationale of this
trial was based on known mitochondrial myopathies, which
involve respiratory enzymes, the known presence of CoQ10 in
respiration, and prior clinical data on CoQ10 and dystrophy.
These results indicate that the impaired myocardial function
of such patients with muscular disease may have some
association with impaired function of skeletal muscle, both of
which may be improved by CoQ10 therapy. The cardiac
improvement was definitely positive. The improvement in
well-being was subjective, but probably real. Likely, CoQ10
does not alter genetic defects but can benefit the sequelae of
mitochondrial impairment from such defects. CoQ10 is the only
known substance that offers a safe and improved quality of
life for such patients having muscle disease, and it is based
on intrinsic bioenergetics.
[Efficiency of ubiquinone and p-oxybenzoic acid in
prevention of E-hypovitaminosis-induced development of
muscular dystrophy]
Kuz'menko IV, Donchenko GV, Kovalenko VN, Gololobov AD,
Tarasova NV
Ukr Biokhim Zh (USSR) Sep-Oct 1981, 53 (5) p73-9
It is shown that E-hypovitaminosis-induced muscular
dystrophy in rabbits is accompanied by a sharp decrease in the
body mass, an increase in the urine creatine-index, a decrease
in the vitamin E and ubiquinone contents in the liver and
skeletal muscle tissues. In the myocardium mitochondria a
decrease in the vitamin E content and an increase in the
ubiquinone content are observed. The activity of
NADH-cytochrome c-, NADH-ubiquinone- and
succinate-ubiquinone-reductase also varies in mitochondria of
the studied tissues. In myocardium organellas a direct
dependence is found between the content of ubiquinone, NADH-
and succinate-ubiquinone-reductase activity and an inverse
one-between its content and the activity of the
NADH-cytochrome c-reductase system. It is established that
p-oxybenzoic acid as well as vitamin E prevents development of
muscular dystrophy and causes changes analogous in direction
in the activity of the ubiquinone-dependent enzymic systems of
mitochondria. Ubiquinone-9 is less efficient in preventing the
development of muscular dystrophy.
Effect of coenzyme Q on serum levels of creatine
phosphokinase in preclinical muscular dystrophy.
Folkers K; Nakamura R; Littarru GP; Zellweger H; Brunkhorst
JB; Williams CW Jr; Langston JH
Proc Natl Acad Sci U S A 1974 May;71(5):2098-102
No abstract.
[Some indices of energy metabolism in the tissues
of mice with progressive muscular dystrophy under the action
of ubiquinone]
Monakhov NK, Obol'nikova EA, Igdal LG, Torosian ZhK, Antelava
NA
Vopr Med Khim (USSR) May 1974, 20 (3) p276-84
Coenzyme Q10 (vitamin Q10) is biosynthesized in the human
body and is functional in bioenergetics, anti-oxidation
reactions, and in growth control, etc. It is indispensable to
health and survival. The first double-blind trial was with
twelve patients, ranging from 7-69 years of age, having
diseases including the Duchenne, Becker, and the limb-girdle
dystrophies, myotonic dystrophy. Charcot-Marie-Tooth disease,
and the Welander disease. The control coenzyme Q10 (CoQ10)
blood level was low and ranged from 0.5-0.84 microgram/ml.
They were treated for three months with 100 mg daily of CoQ10
and a matching placebo. The second double-blind trial was
similar with fifteen patients having the same categories of
disease. Since cardiac disease is established to be associated
with these muscle diseases, cardiac function was blindly
monitored, and not one mistake was made in assigning CoQ10 and
placebo to the patients in both trials. Definitely improved
physical performance was recorded. In retrospect, a dosage of
100 mg was too low although effective and safe. Patients
suffering from these muscle dystrophies and the like, should
be treated with vitamin Q10 indefinitely.
Free radicals, lipid peroxides and antioxidants in
blood of patients with myotonic dystrophy.
Ihara Y, Mori A, Hayabara T, Namba R, Nobukuni K, Sato K,
Miyata S, Edamatsu R, Liu J, Kawai M
Clinical Research Institute, National Minamiokayama Hospital,
Okayama, Japan.
J Neurol. 1995 Feb. 242(3). P 119-22
We studied the levels of free radicals, lipid peroxides and
antioxidants, as well as superoxide dismutase (SOD) activity
in the blood of six patients with myotonic dystrophy (MyD)
(mean age 52.8, SD 5.0 years) and seven controls (mean age
48.8, SD 6.3 years). Electron spin resonance was used to
assess the free radicals by the spin-trapping method using
5,5-dimethyl-1-pyrroline-1-oxide. The levels of C centre
radical (P < 0.05) and H radical (P < 0.05) in blood
from the six MyD patients were significantly higher than those
in the seven controls. The SOD activities in red blood cells
and serum from the six MyD patients showed no significant
difference from those in the seven controls. The serum lipid
peroxide concentration was increased in five of the MyD
patients and tended to increase further as the disease
progressed. The serum vitamin E level was low in two patients
and in the low normal range in three. Serum coenzyme Q10 was
decreased in four patients. The serum selenium level was
decreased in two patients and that of serum albumin was
decreased in three. Therefore we conclude that increased
levels of free radicals and lipid peroxides and decreased
antioxidant levels play an important role in the pathogenesis
of MyD.
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