By S. Harvey Mudd (auth.), Harold H. Draper (eds.)
Volume four of Advances in dietary learn displays the elevated significance that lately has been connected to foodstuff in lots of fields of medical medication. This heightened curiosity in foodstuff stems from the demonstration that the consumption of particular nutrition could have far-reaching effects, not just for regular metabolism, but in addition for metabolic strategies affecting scientific or subclinical sickness. Conversely, many disorder states were proven to have formerly unrecognized results on nutrient functionality and metabolism. as well as issues of visible relevance to human medical food, this quantity comprises chapters facing the nutrients of cells grown in tradition and of species which can supply insights into dietary issues of guy. To gether with its predecessors, quantity four presents graduate scholars and estab lished investigators with authoritative bills of the prestige of analysis on a variety of themes of present curiosity in experimental and medical foodstuff. vii Contents bankruptcy 1. Vitamin-Responsive Genetic Abnormalities .......... . S. Harvey Mudd 1. Introduction.............................................. 1 2. historic point of view ...................................... 2 three. Genetic Abnormalities at present identified to Be nutrition 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 engage with the Cofactor varieties of supplementations. . . . . . . . . . . . . . . . . . sixteen . . . . . .
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Extra resources for Advances in Nutritional Research
Clin. Esp. 106:448. , 1960, Selective vitamin B12 malabsorption and proteinuria in young people, Acta Med. Scand. 167:289. Gravel, R. , Mahoney, M. , Ruddle, F. , and Rosenberg, L. , 1975, Genetic complementation in heterokaryons of human fibroblasts defective in cobalamin metabolism, Proc. Natl. Acad. Sci. A. 72:3181. , Neiman, P. , Canellos, G. , 1971, Neonatal megaloblastic anemia due to inherited transcobalamin II deficiency in two siblings, N. Engl. J. Med. 285:1163. , 1975, The Principles of Human Biochemical Genetics, North-Holland, Amsterdam.
Several findings make these quantitative relationships understandable. , 1970b) have shown that normal humans possess the capacity to metabolize daily at least 60 mmole of homocysteine through the cystathionine J3-synthase step, a capacity which is large relative to the need imposed by the normal dietary intake of about 10 mmole of methionine. Cystathionine J3-synthase-deficient patients on basal diets, however, could convert only about 3 mmole of methionine sulfur to sulfate, an amount insufficient to metabolize the normal daily intake.
1 % of control in the two experimental series. In contrast, among sibships not responsive to Bs, only four had detected residual activity of cystathione f3-synthase, whereas 13 had no detected activity. Thus, there are good correlations between the presence of a small residual activity of cystathionine f3-synthase and Bs responsiveness on the one hand, and between the lack of residual activity and Bs nonresponsiveness on the other. With the advent of the use of the fibroblast system, liver biopsy for study of cystathionine f3-synthase deficient patients has largely ceased.
Advances in Nutritional Research by S. Harvey Mudd (auth.), Harold H. Draper (eds.)