Intestinal β-Galactosidases in Adult Low Lactase Activity1 and in Congenital Lactase Deficiency

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Abstract
Three different β-galactosidases in the human small intestine have been partially purified and characterized. Quantitative methods, applicable on single jejunal biopsies, were then elaborated to study the enzymatic alteration in adult low lactase activity and in congenital lactase deficiency. The activity of brush-border lactase was 10-20 times reduced in adults with specific low lactase activity. The residual enzyme had the same properties as in adults with persistently high lactase activity. In three patients with congenital lactase deficiency, on the other hand, no brush-border lactase at all was demonstrable, while a very weak activity was found in a fourth patient. The activities of lysosomal acid β-galactosidase and cytosol hetero-β-galactosidase were not altered.

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