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( ). . . . 21.3.

1, ( ). - -1- (-) (-) -1-. ( 2), -1---, - -. ( 3) . , -, . , , NAD . , - -1- ( 4), , .

3 , , , , . , (), .

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. 21.3. .

(. )

. 21.4. . UDP- . . TDP- TDP-N-.

-. , .

, , 1, 2, 3 . 21.3. (2). . (). . : -1-, . , .

-1-- ( 2), , ( 3) , UDP-

. , , ( ). , , . , 50% ( ) ; . , , .

() (. 21.4)

(. . 54), (, , . . 15) (. . 54). , -, ( ) -9- . , , N- (NeuAc). . 21.4; : (1) ; -6- -6-, ; (2) N- , -; (3) N--6- N--6-; (4) NeuAc -6- ; (5) UDP-N- (UDPGlcNAc) UDP-N- (UDP-GalNAc); (6) , UDPGlcNAc, UDPGalNAc CMPNeuAc.

Brown D.H., Brown . I. Some inborn errors of carbohydrate metabolism. Page 391. In: MTP International Review of Science, Vol 5, Whelan W.J. (ed.), Butterworth, 1975. Dickens F., Randel P.J., Whelan W.J. (ed.). Carbohydrate Metabolism and Its Disorders, 2 vols, Academic Press, 1968. Huijing F. Textbook errors, Galactose metabolism and galactosemia, Trends Biochem. Sci., 1978, 3, N 129.

James H. M. et al. Models for the metabolic production of oxalate from xylitol in humans, Aust. J. Exp. Biol. Med. Sci., 1982, 60, 117.

Kador P. F., Akagi Y., Kinoshita J. H. The effects of aldose reductase and its inhibition on sugar cataract formation, Metabolism, 1986, 35, 15.

Macdonald /Vrana A. (eds). Metabolic Effects of Dietary Carbohydrates, Karger, 1986.

Randle P.J., Steiner D.F., Whelan W.J. (eds). Carbohydrate Metabolism and Its Disorders, Vol 3, Academic Press, 1981.

Sperling O., de Vries A. (eds). Inborn Errors of Metabolism in Man, Karger, 1978.

Stanbury J. B. et al. (eds). The Metabolic Basis of Inherited Disease, 5th ed. McGraw-Hill, 1983.

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