Advances in Nutritional Research by S. Harvey Mudd (auth.), Harold H. Draper (eds.)

By S. Harvey Mudd (auth.), Harold H. Draper (eds.)

Volume four of Advances in dietary learn displays the elevated significance that lately has been hooked up to foodstuff in lots of fields of scientific medication. This heightened curiosity in food stems from the demonstration that the consumption of particular nutrition can have far-reaching results, not just for regular metabolism, but in addition for metabolic strategies affecting scientific or subclinical disorder. Conversely, many affliction states were proven to have formerly unrecognized results on nutrient functionality and metabolism. as well as issues of noticeable relevance to human medical food, this quantity includes chapters facing the food of cells grown in tradition and of species which may offer insights into dietary problems of guy. To­ gether with its predecessors, quantity four offers graduate scholars and estab­ lished investigators with authoritative money owed of the prestige of analysis on a number of subject matters of present curiosity in experimental and medical meals. vii Contents bankruptcy 1. Vitamin-Responsive Genetic Abnormalities .......... . S. Harvey Mudd 1. Introduction.............................................. 1 2. old point of view ...................................... 2 three. Genetic Abnormalities at present recognized to Be diet Responsive. three four. Mechanisms Underlying diet Responsiveness. . . . . . . . . . . . . . . . 7 4.1. Defects within the Processing of a nutrition ahead of Its interplay in Cofactor shape with a selected Apoenzyme ........ 7 4.2. Defects particularly Apoenzymes that have interaction with the Cofactor sorts of supplements. . . . . . . . . . . . . . . . . . sixteen . . . . . .

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1954, Pyridoxine dependency: Report of a case of intractable convulsions in an infant controlled by pyridoxine, Pediatrics 13: 140. , 1960, Idiopathic chronic megaloblastic anemia in children, Acta Paediatr. Scand. J119:1. , 1975, Studies on new intracellular proteases in various organs of rat. I. Purification and comparison of their properties, Eur. 1. Biochem. 52:37. , Lee, S. , and Cooper, B. , 1972, Vitamin 8 12 malabsorption due to biologically inert intrinsic factor, N. Engl. 1. Med. 287:425.

1980b). In all these situations, the antioxidant action of vitamin E is thought to prevent damage that would otherwise occur because of lack of normal amounts of reduced glutathione. 5. Significance of Vitamin-Responsive Genetic Conditions Most of the genetic conditions discussed in this chapter are rare, and one may understandably wonder whether they have meaning for more everyday situations. In the opinion of the author, there are two major reasons why nutritionists may wish to be aware of these conditions.

For example, Brunette and co-workers (1972) showed that a patient with lactic acidosis resulting from a defect in pyruvate carboxylase was responsive to thiamine. Thiamine pyrophosphate is not a cofactor for the defective enzyme but is a cofactor for another enzyme that disposes of pyruvate, pyruvate dehydrogenase. In assays with intact leucocytes from both control subjects and the patient with pyruvate carboxylase deficiency, addition of thiamine to the medium enhanced the flux through the pyruvate dehydrogenase step.

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