Tocopherol deficiency

Gordon, 11. H Nilowsky, H. M., and Cornblath, M. (1955). Studies of tocopherol deficiency in infants and children. Am. f. Dis. Ctiild. 90,669-681-Cotoda, T, Arita, M., Aral, H Inoue, K Yokota, T., Fukuo, Y Yazaki, Y, and Yamada, N. (1995). Adult-onset spinocerebellar dysfunction caused by a mutation in the gene for the n-tdcopherol-transfer protein. N. Engi. j. Med. 333, 1313-1318. 

Nickander KK, Schmelzer JD, Rohwer DA. Low PA. Effect of alpha-tocopherol deficiency on indices of oxidative stress in normal and diabetic peripheral nerve. J Neurol Sci 1994 126 6-14. 

In a group of five infants who received the same formula plus a daily oral supplement of 100 mg of mixed tocopherols (60 mg of a-to-copWol), the plasma tocopherol rose from 0.35 0.05 (SE) mg/100 ml at birth to 0.79 0.17 (SE) mg/100 ml at 4 weeks. The susceptibihty to peroxide hemolysis remained low and constant, 10 3 (SE) at birth and 11 6 (SE) at 4 to 5 weeks. Infants in this group had no clinical symptoms suggesting tocopherol deficiency. 

Hofius, D., Hajirezaei, M.R., Geiger, M., Tschiersch, H., Melzer, M., and Sonnewald, U., RNAi-mediated tocopherol deficiency impairs photoassimilate export in transgenic potato plants. Plant. Physiol. 135 (3), 1256-1268, 2004. 

F. Erythrocyte Survival Time during Tocopherol Deficiency. 550... 

F. Ekythrocyte Survival Time duri.vg Tocopherol Deficiency... 

Using Cr -labeled cells, a shortened erythrocyte life span was found in newborn premature infants (Kaplan and Hsu, 1961). The susceptibility of their erythrocytes to in vitro hemolysis in hydrogen peroxide suggests a possible relationship to their decreased survival in vivo. Indeed, decreased survival has been observed in the vitamin E-deficient monkey (Marvin et al., 1959) although the normal survival in vitamin E-defident rats suggests a species variability (Goldbloom, 1960). The results in humans are inconclusive. A decreased red cell life span was found in adult volunteers on a tocopherol-deficient diet high in polyunsaturated lipids (Horwitt et al., 1961), but survival was normal in a patient with cystic fibrosis of... 

In addition to the studies undertaken at El n in which human a-tocopherol deficiency has been produced artificially, many cases of naturally occurring tocopherol deficiency have been described in the human These were associated with abnormal diets or, more commonly, with fat absorption defects from various causes. 

Some of the patients showed other biochemical, morbid anatomical and clinical evidence of tocopherol deficiency hydrogen peroxide hemolysis, creatinuria, ceroid pigmentation (Pappenheimer and Victor, 1946 Adlers-burg and Schein, 1946 Tuerdy et al., 1949 Blanc et al., 1958 Kerner and Goldbloom, 1960, Froelich et al., 1961) cerebellar lesions (Horwitt and... 

Bailey, 1959) and necrosis of striated muscle. The striated muscle lesions were first found by Bramwell and Muir (1907-1908) and have since been found by Blanc et al. (1958), Oppenheimer (1956), Weinberg et al., (1958). They are similar to those found in animals with tocopherol deficiency muscular dystrophy. 

Richards et al. (1957), however, could find no correlation between the degree of peroxide hemolysis and the intensity of physiological jaundice in the newborn, and Goldbloom (1960) found normal red cell survival in tocopherol-deficient rats and in a 2-year-old boy with fibrocystic disease of the pancreas and persistently low serum tocopherol levels. 

In view of these animal studies showing effects on factors concerned with tissue metabolism, viz, adenosine triphosphate (ATP), coenzyme A, and ubiquinone, it should be possible to show differences between the responses of normal and tocopherol deficient tissues to oxygen. 

We measured plasma vitamin E in five subjects with TCHAD deficiency who were prescribed a multivitamin with vitamin E and found four of the five had biochemical a-tocopherol deficiency (Fig. 23.10). 

In vitamin E-deficient rabbits, muscular dystrophy can be corrected by the administration of a-tocopherol but not selenium. Tocopherol-deficient monkeys develop an anemia that is reversed by the administration of vitamin E or coenzyme Q chromanols, but not by coenzyme chromanols or selenium. 

Dietary deficiency of vitamin E also leads to degeneration of the seminiferous tubules. Changes in the fatty acid composition of testes of rats receiving a vitamin E-deficient diet have been reported. An increase in arachidonic acid and in 22 4n-6 and a decrease in 22 5n-6 were found by Bieri Andrews (1964), who suggested that there might be a metabolic block in the conversion of 20 4 to 22 5 in vitamin E-deficiency. Witting et al. (1967) postulated that 22 5n-6 may be destroyed by peroxidation in the testis of the tocopherol-deficient rat, while Carney Walker (1971)... 

Draper, H. H, and Scallany, A. S. (1958) Action of NN diphenyl-p-phenylene diamine in tocopherol deficiency diseases, Proc, Soc. Exp, Biol. Med., 99 739. 

Although lipoic acid has been found in numerous biological tissues, reports on its concentrations in foods are scarce. Lipoic acid is detectable in wheat germ (0.1 ppm) but not in wheat flour. It has been detected in bovine liver kidney and skeletal muscle. Oral administration of lipoic acid (1.65 g/kg fed) to rats for five weeks resulted in elevated levels of the thiol in liver, kidney, heart, and skin. When lipoic acid was added to diets lacking in vitamin E, symptoms typical of tocopherol deficiency were not observed suggesting that lipoic acid acts as an antioxidant in vivo. However, lipoic acid was not capable of recycling vitamin E in vivo, as determine by the fact that a-tocopherol concentrations are not elevated by dietary lipoic acid in vitamin E deficient rats. 

Other naturally occurring tocopherols are the y-, and 6-tocopherols they are short one or two methyl groups on the aromatic ring. Concerning tocopherol deficiency, see Table XIX, Chapt. XXII. ... 

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