Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.26 (invertase)
4,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

7 infants, aged 5 weeks to 11 months, with clinically documented intolerance to cow's milk protein, chronic diarrhea, and failure to thrive, underwent small intestinal (peroal, suction) biopsy before and after withdrawal of milk proteins. Mucosal specimens were examined by light microscopy and assayed for disaccharidase activities. In all patients, moderate to severe mucosal changes were presented, associated with marked inflammation of lamina propria and damages to the brushborder. Disaccharidase activities (lactase, sucrase, maltase and palatinase) were markedly depressed in all. Follow-up biopsies were obtained in 6 infants, after 3-5 months on a milk-protein-free diet. At the time of the second biopsy, the disaccharidase activities had risen significantly and histologic improvement had occurred in each instance. In infancy, intestinal mucosal lesions due to intolerance to cow's milk protein are histologically indistinguishable from those seen in gluten-sensitive enteropathy and are associated with marked secondary disaccharidase deficiencies. Following therapy, the activity of the disaccharidases become normal or near normal prior to the complete morphologic recovery of the small intestinal mucosa.
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PMID:Disaccharidase deficiency in infants with cow's milk protein intolerance. Response to treatment. 62 28

Follow-up studies on 36 children, in whom celiac disease (gluten-sensitive enteropathy) was established by gluten challenge, were carried out after management on gluten-free diets for a mean of six years. Evaluations included measurement of height and weight, which for the group approximated normal distributions, and histologic examination of the duodenal or jejunal mucosa. Mucosal morphology was regarded as normal in 16, and there were minimal changes in 20. Epithelial cell height was within the normal range in all the children. Interepithelial lymphocytes were within normal range in the majority and lymphoid cells in the lamina propria were not different from those in control subjects. Mucosal lactase was significantly lower in patients than in control subjects in the duodenum and the jejunum, whereas sucrase and alkaline phosphatase values were significantly lower in the jejunum but not in the duodenum. Low content of mucosal lactase and increased numbers of interepithelial lymphocytes may be sensitive indicators of persisting ingestion of gluten in mucosa that is otherwise normal or approximately so in appearance.
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PMID:Mucosal recovery in treated childhood celiac disease (gluten-sensitive enteropathy). 95 66

Lactase deficiency, manifested clinically by lactose malabsorption, is often the only biochemical evidence of a residual disturbance of jejunal mucosal function after Escherichia coli enteropathy in the infant. Villous morphology is usually normal. A sustained depression of the processes of biochemical differentiation of lactase biosynthesis has been postulated to explain similar states of lactase deficiency, but a possible influence of altered epithelial cell turnover on the mucosal lactase levels has not been investigated. In ten infants with a residual lactose malabsorption, after E. coli infection, jejunal cell renewal activity and disaccharidase activities were studied by analysis of the exfoliated cells collected by lumenal perfusion. Significant increases in DNA and protein exfoliation and in the brush border activities of sucrase and lactase were observed during recovery from the malabsorptive disturbance. DNA and protein efflux increased almost linearly during a 20-day period. Lactase was initially four times more deficient than sucrase activity in the exfoliated cells. Both enzyme activities increased at almost identical rates. Therefore, it took longer for lactase activity to return to normal levels. The lactase/sucrase ratios approached normal at the end of the 20-day period. The changes in the exfoliating levels of the two enzymes, when analysed in relation to the increases in cell renewal activity, suggested a relationship between sucrase and lactase levels and cell age.
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PMID:Intestinal exfoliated cells in infant diarrhoea: changes in cell renewal and disaccharidase activities. 104 54

Clinical differences between the two human intestinal mucosal folate conjugases were assessed by measurement of their activities in normal individuals and in patients with chronic diarrhea of differing causes. Intracellular folate conjugase (ICFC) was 15-fold more active than brush border folate conjugase (BBFC) in jejunal mucosa from seven obese patients undergoing elective gastric bypass surgery. The activity of ICFC was similar among normal volunteers and patients with diarrhea of unknown origin (DUO), gluten-sensitive enteropathy (GSE), inflammatory bowel disease (IBD), and the short bowel syndrome (IBD-SBS). By contrast, BBFC, sucrase, and lactase were decreased significantly in GSE, and BBFC was increased in IBD-SBS. The activity of BBFC correlated with lactase and with sucrase in the normal subjects and in patients with DUO, whereas no correlations were found with the activity of ICFC in any group. Our clinical studies confirm that ICFC and BBFC are different enzymes. ICFC is not affected by intestinal disease, whereas the activity of jejunal BBFC, like that of other brush border enzymes, is decreased by mucosal injury and is also capable of adapting to distal small intestinal disease or surgical resection.
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PMID:Clinical studies of intestinal folate conjugases. 308 71

There is a reported association between administration of prenatal glucocorticoids and a decreased incidence of necrotizing enterocolitis in human infants. In rats, the degree of ischemic bowel disease correlates negatively with intestinal diamine oxidase (E.C. 1.4.3.6) activity. Since the administration of hydrocortisone, thyroxine, or phenobarbital to newborn rat pups affects the development of intestinal enzymes, we were interested in knowing whether hydrocortisone, thyroxine, or phenobarbital specifically affect intestinal diamine oxidase activity. We injected rat pups with hydrocortisone sodium succinate, 1-thyroxine pentahydrate, sodium salt, sodium phenobarbital, or the control solution on days 4, 6, 8, or 10 of life (phenobarbital, days 3, 5, 7, or 9). Pups were injected 3 days consecutively (phenobarbital, 4 days), and all were sacrificed on days 7, 9, 11, and 13. Intestinal diamine oxidase and intestinal invertase (E.C. 3.2.1.26) activities were measured. Invertase was used as a control enzyme because it is known to be induced by glucocorticoid hormones. We found that the hydrocortisone-injected pups had 10-fold higher specific activity of invertase than the saline-injected animals. Diamine oxidase activity was significantly higher in the group receiving hydrocortisone and sacrificed on days 7, 9, and 11. Enzyme activity in both the hydrocortisone-injected and saline-injected groups was equal on day 13, as was enzyme activity on all days in the thyroxine-injected and sodium hydroxide-injected groups, and the phenobarbital-injected and the saline-injected groups. Our results suggest that diamine oxidase activity may be induced by hydrocortisone, but is not affected by thyroxine or phenobarbital.
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PMID:The effect of hydrocortisone, thyroxine, and phenobarbital on diamine oxidase activity in newborn rat intestine. 310 23

To assess the course of recovery of gluten sensitive enteropathy in adults, histological and functional recovery was studied in 22 patients, aged 20-79 years. Biopsy specimens taken at the time of diagnosis were studied in 20; after adhering to a gluten free diet for nine to 19 (mean 14) months in 14; and after adhering to the same diet for 24-48 (mean 34) months in 10 patients. Histological recovery was assessed morphometrically in the proximal jejunum. Mucosal linings significantly improved over time, but did not completely return to normal with a gluten free diet: at diagnosis the surface: volume ratio was 22% of normal, increasing to 48% and 66% after nine to 19 and 24-48 months, respectively, of a gluten free diet. Disaccharidase activities progressively increased. After 24-48 months maltase, sucrase, and isomaltase had returned to normal in the proximal jejunum; they were still significantly decreased in the distal duodenum. Duodenal and jejunal lactase activities were both below normal after 24 to 48 months. It is concluded that recovery of the intestinal mucosa of adults with gluten sensitive enteropathy during a gluten free diet continues beyond nine to 19 months and is still incomplete after two to four years. The recovery of disaccharidase activities extends from the distal to the proximal part of the small intestine, and is aligned to histological recovery.
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PMID:Slow and incomplete histological and functional recovery in adult gluten sensitive enteropathy. 317 Jul 77

Fifty two children in whom coeliac disease was confirmed by persistent enteropathy while they were taking gluten were monitored to assess the effects of compliance with a gluten free diet (GFD). Between the ages of 17.8 and 18.5 years height (in 45 patients followed up for a mean of 14.9 years) and weight (in 43 followed up for a mean of 15.2 years) were significantly lower in those complying poorly with a GFD compared with those complying well. Of the 37 patients still attending the clinic after a mean of 25 years, having been followed up for a mean of 18.4 years, 16 who had complied well with the diet had normal or only slightly abnormal mucosal morphology whereas all 10 who had not complied had abnormal morphology. In these 10 lactase, sucrase, and alkaline phosphatase activities were significantly less than values in those who complied well. Mucosal sucrase and alkaline phosphatase activities in those who complied well were no different from those in a control population, whereas lactase activity was significantly lower. It is concluded that failure to comply with a GFD during childhood results in decreased adult stature and in persisting active enteropathy with depressed brush border enzyme activity.
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PMID:Compliance with gluten free diet in coeliac disease. 363 17

Mucoid enteropathy was induced experimentally by ligation of the cecum, and the activities of mucosal disaccharidases and alkaline phosphatase were measured at different locations along the small intestine of the sick and control rabbits. In the duodenum of rabbits with mucoid enteropathy, the activity of acid beta-galactosidase II was elevated and hetero beta-galactosidase declined. In the jejunum, the activities of lactase, acid beta-galactosidase I and II, hetero beta-galactosidase, trehalase, sucrase and alkaline phosphatase were significantly lower in animals with mucoid enteropathy. In the ileum, acid beta-galactosidase II, hetero beta-galactosidase, maltase, trehalase, sucrase and alkaline phosphatase showed decreased activity in rabbits with mucoid enteropathy.
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PMID:Intestinal disaccharidase and alkaline phosphatase activities in experimental rabbit mucoid enteropathy. 409

A naturally occurring enteropathy was identified in Irish setter dogs and wheat-sensitivity was demonstrated in a litter bred from two of the affected animals. The morphological and biochemical features of this enteropathy are described and compared to coeliac disease in man. Affected animals comprised 10 dogs that presented with poor weight gain or weight loss, with or without diarrhoea. Exocrine pancreatic function was normal and culture of duodenal juice demonstrated no marked bacterial overgrowth. Serum vitamin B12 concentrations were unaltered, but in some cases low serum and erythrocyte folate concentrations and reduced xylose absorption provided indirect evidence for proximal small intestinal disease. Examination of peroral jejunal biopsies revealed patchy morphological changes within individual animals, comprising predominantly partial, but in one case subtotal, villous atrophy. Brush border enzymes were selectively altered: the specific activities of alkaline phosphatase, leucyl-2-naphthylamidase and of zinc-resistant alpha-glucosidase were reduced by approximately 40 per cent, while activities of maltase, sucrase, lactase and gamma-glutamyl transferase were unaltered. Activity of a lysosomal enzyme was increased and there was evidence for enhanced lysosomal fragility. The activity of malate dehydrogenase, with a dual mitochondrial and cytoplasmic localisation, was decreased but there were no changes in the activities of marker enzymes for basal-lateral membranes, endoplasmic reticulum or peroxisomes. These findings, particularly the specific biochemical abnormalities, were comparable to those in partially treated coeliac disease in man; however, a specific role for wheat in the pathogenesis of the disease has yet to be defined.
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PMID:Morphological and biochemical studies of a naturally occurring enteropathy in the Irish setter dog: a comparison with coeliac disease in man. 652 28

The subcellular biochemical features of a naturally occurring enteropathy in the dog associated with bacterial overgrowth have been examined. Affected animals comprised a group of 10 German Shepherd dogs with raised serum folate and reduced vitamin B12 concentrations, mild steatorrhoea, reduced xylose absorption, and normal exocrine pancreatic function. Culture of duodenal juice showed bacterial overgrowth with mixed flora, most frequently including enterococci and Escherichia coli. Examination of peroral jejunal biopsies revealed predominantly minimal histological but distinct biochemical abnormalities in the mucosa. The specific activity of alkaline phosphatase was decreased, isopycnic density gradient centrifugation showing a marked loss particularly of the brush border component of enzyme activity. In contrast, gamma-glutamyl transferase activity was enhanced in brush border fragments of slightly increased modal density, but there were no changes in the activities of the carbohydrases, zinc-resistant alpha-glucosidase, maltase, sucrase, and lactase or of the peptidase, leucyl-2-naphthylamidase. Activities of lysosomal enzymes were increased and there was evidence for enhanced lysosomal fragility and mitochondrial disruption. The activities and density gradient distributions of marker enzymes for basal-lateral membranes, endoplasmic reticulum and peroxisomes were essentially unaltered. These findings show that bacterial colonisation of the proximal small intestine may be associated with specific alterations in microvillus membrane proteins and provide biochemical evidence for intracellular damage to the enterocytes.
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PMID:Biochemical changes in the jejunal mucosa of dogs with a naturally occurring enteropathy associated with bacterial overgrowth. 674 19


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