Inherited Disorders of Carbohydrate Metabolism: Monograph by H. G. Hers (auth.), D. Burman, J. B. Holton, C. A. Pennock

By H. G. Hers (auth.), D. Burman, J. B. Holton, C. A. Pennock (eds.)

The 16th annual symposium of the Society for the examine of Inborn mistakes of Metabolism was once held in Bristol from twelfth to 14th July, 1978. approximately 25 invited audio system and one hundred fifty members got here from many elements of Europe and North the United States to contemplate the subject, 'Inherited problems of Carbohydrate Metabolism'. even if a few features of those problems have shaped a part of the programme of prior symposia equipped via the Socie­ ty, this was once the 1st try to speak about them in a scientific demeanour. the topic, carbohydrate issues, embraces either favourite and good documented stipulations and a few lesser identified points of genetic affliction. In all of those there continues to be a lot to be learnt approximately scientific and laboratory analysis, remedy, biochemical screening and pathogenesis. hence one goal of the Society, to mix medical and clinical curiosity, can hardly were higher accomplished in one symposium. because the programme integrated ailments from six assorted components of vehicle­ bohydrate metabolism and contained such a lot of unique audio system, it truly is most unlikely to spotlight the extra vital features of this symposium inside of a brief house. each one part made a amazing contribution to wisdom and, while time used to be to be had, energetic discussions ensued which were recorded within the e-book. although, we want to point out our specified lectures, simply because they understand humans to whom the Society owes greatly. The Milner lecture has been given for the previous 6 years as a tribute to Mr J.

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Inherited Disorders of Carbohydrate Metabolism: Monograph based upon Proceedings of the Sixteenth Symposium of The Society for the Study of Inborn Errors of Metabolism

The 16th annual symposium of the Society for the research of Inborn error of Metabolism used to be held in Bristol from twelfth to 14th July, 1978. approximately 25 invited audio system and a hundred and fifty contributors got here from many elements of Europe and North the USA to contemplate the subject, 'Inherited issues of Carbohydrate Metabolism'.

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Additional info for Inherited Disorders of Carbohydrate Metabolism: Monograph based upon Proceedings of the Sixteenth Symposium of The Society for the Study of Inborn Errors of Metabolism

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6. Three distinct lactases (p-galactosidases) have been identified 7- 11 , a neutral brush border lactase, a neutral cytoplasmic hetero ,B-galactosidase and an acid lysosomal ,B-galactosidase. 1 hydrolysis Structure of components of starch and enzymes responsible for their importance in the physiology of lactose absorption. All the a-glucosidases, except trehalase, hydrolyse maltose and thus can be considered as maltases; indeed as much as 75% of mucosal maltase activity can be accounted for by sucrase.

Separation and characterization of three forms of an acid ,B-galactosidase. Biochem. , 121, 299 10. , Fric, P. and Jode, J. (1972). Histochemische Befunde in der Dlinndarmschleimhaut bei St6rungen der Kohlenhydratabsorption. Dtsch. Z. Verdau. , 32, 163 II. -G. and Dahlqvist, A. (1974). Intestinal ,B-galactosidases in adult low lactase activity and in congenital lactase deficiency. Enzyme, 18, 84 12. Conklin, K. , Yamashiro, K. M. and Gray, G. M. (1975). Human intestinal sucrase-isomaltase. Identification of free sucrase and isomaltase and cleavage of the hybrid into active distinct subunits.

24. 25. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35 Honegger, P. and Semenza, G. (1973). Multiplicity of carriers for free glucalogues in hamster small intestine. Biochim. Biophys. Acta, 318, 390 Debnam, E. S. and Levin, R. J. (1976). Influence of specific dietary sugars on the jejunal mechanisms for glucose, galactose, and a-methyl glucoside absorption: evidence for multiple sugar carriers. , Caspary, W. F. and Crane, R. K. (1973). Studies on the transport of glucose from disaccharides by hamster small intestine in vitro.

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