Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.20 (alpha-glucosidase)
 4,237 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lactase, maltase, sucrase, and alkaline phosphatase activities were determined in the intestinal mucosa from 3 locations in the small intestine and 4 locations in the large intestine 1 year after extensive large-colon resection (group 1; n = 5) and 1 year after sham operation (group 2; n = 3) in horses. Lactase, maltase, and sucrase activities were similar (P greater than 0.05) between group-1 and group-2 horses in all locations measured in the intestinal tract. Alkaline phosphatase activity in the remaining large colon of group-1 horses was significantly (P less than 0.05) greater than the activity in the large colon of group-2 horses. Decreased apparent digestion of phosphorus and a negative phosphorus balance are persistent features of large-colon resection in horses. Increases in alkaline phosphatase activity in the remaining colon of horses with extensive large-colon resection may be a specific functional adaptive mechanism that attempts to counteract the derangements in phosphorus metabolism.
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PMID:Alteration of intestinal enzyme activities associated with extensive large-colon resection in horses. 211 42

Mesenteric vascular occlusion and intestinal obstruction are difficult-to-diagnose medical emergencies. We evaluated a large panel of biochemical markers as diagnostic and prognostic indicators in a rat model of intestinal infarction and partial, complete, and strangulated intestinal obstruction. After intestinal infarction and obstruction, laboratory data are distinctly abnormal. Serum urea nitrogen dramatically increased in all groups, but most rapidly in the groups with infarction and strangulated obstruction. Inorganic phosphorus proved to be a sensitive indicator of infarction, but less so for any form of obstruction. While all members in the infarct group demonstrated significant increases in the aminotransferases, creatine kinase, and alkaline phosphatase, such increases in the groups with obstruction were less pronounced. Serum maltase assays revealed decreasing activities in all members of the groups with complete and strangulated obstruction, but in only 17% of the rats with partial obstruction. Serum maltase activity increased from abnormally low values after surgery to abnormally high values in the six animals that recovered from partial intestinal obstruction. The proportion of hexosaminidase A (of total beta-N-acetylhexosaminidase, EC 3.2.1.30) was generally abnormal in rats with complete and strangulated obstruction.
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PMID:Acute intestinal infarction or obstruction: search for better laboratory tests in an animal model. 296 10

Maternal vitamin D deficiency has been shown to lead to reduced body weights in developing rat pups. To evaluate the effects of vitamin D deficiency alone both in dams and pups during the perinatal age on the ontogeny of gastrointestinal enzymes, female weanling rats (3 weeks of age) were divided into three groups. Groups I and II were fed a control (vitamin-D-replete) diet. Group II were fed a vitamin-D-deficient diet. Six weeks afterward they were mated with normal male rats while continuing on their respective diets until sacrifice. Only rats that delivered their pups on the same day from each group were brought into the study. Litter sizes of groups I and II were adjusted to 10, while group III was adjusted to 13 such that the rate of growth paralleled that of group II. At 19 days after birth, all dams and pups were sacrificed. There were no differences in the calcium and phosphorus contents in breast milk obtained from dams of each group. The serum calcium concentration of pups from group II (vitamin-D-deficient) was lower than the other groups. Body weights of pups from groups II and III were significantly lower than those of group I. The mucosal weight, total mucosal protein, mucosal DNA, sucrase, and maltase activities from groups II and III were similar, but lower than group I. Pancreatic weight, total pancreatic protein, DNA, amylase, and lipase activities from groups II and III were also similar, but lower than group I. Vitamin D deficiency was confirmed in both dams and pups from group II.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Vitamin D deficiency, pancreatic and small intestinal enzyme development in rats. 320 79

We have recently shown that lamellar body fractions purified from human lung contain a distinct acid alpha-glucosidase distinguishable from lysosomal acid alpha-glucosidase in that it does not cross-react with antibodies raised against the lysosomal enzyme and does not bind to concanavalin A (De Vries, A.C.J., Schram, A.W., Tager, J.M., Batenburg, J.J. and Van Golde, L.M.G. (1985) Biochim. Biophys. Acta 837, 230-238). In order to study the relationship between the non-concanavalin A-binding alpha-glucosidase and lamellar bodies more closely a method was developed for the further purification of the organelles. A purified lamellar body preparation isolated from human lung homogenate by discontinuous sucrose density centrifugation was subjected to gel filtration with Sepharose 4B followed by Percoll density gradient centrifugation, which yielded a lamellar body preparation with a phospholipid phosphorus/protein ratio of 12.57 +/- 0.38 (mumol/mg) (n = 3) as compared to a ratio of 3.34 +/- 0.16 (mumol/mg) (n = 3) in the sucrose density gradient preparation. Concomitantly there was a 3.3 +/- 0.1 (n = 3)-fold enrichment in the content of total acid alpha-glucosidase and a 3.2 +/- 0.1 (n = 3) -fold enrichment of non-concanavalin A-binding acid alpha-glucosidase. The new purification method removes adhering proteins without changing the phospholipid composition. During the successive purification steps the concanavalin A-sensitive and -insensitive alpha-glucosidases remained fully lamellar body fraction associated. Differences between a lysosome-enriched fraction and a lamellar body preparation at varying stages of purification with respect to the ratio between soluble acid hydrolases and the membrane-associated lysosomal enzyme glucocerebrosidase indicate that the purified lamellar bodies were not contaminated with lysosomes. The absence of lysosomes in the purified lamellar body fraction was confirmed by experiments with the weak base glycyl-L-phenylalanine-beta-naphthylamide, which is an artificial substrate for the lysosomal enzyme cathepsin C and brings about lysis of lysosomes. Morphological examination by electron microscopy endorses the absence of contaminating vesicles and organelles and showed a structural integrity of the lamellar bodies in the final preparation. The improved isolation procedure strongly suggests that the concanavalin A-insensitive acid alpha-glucosidase is endogenous to lamellar bodies and supports our earlier idea that it can be used as a lamellar body-specific marker enzyme. In addition, the experiments show that lamellar bodies free of lysosomes contain a spectrum of lysosomal-type enzymes.
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PMID:An improved procedure for the isolation of lamellar bodies from human lung. Lamellar bodies free of lysosomes contain a spectrum of lysosomal-type hydrolases. 368 11

We explored whether glucocorticoid administration, a known stimulus of renal gluconeogenesis (GNG), could decrease avid inorganic phosphate (Pi) reabsorption in rats stabilized on low-phosphorus diet (LPD). Rats adapted to LPD were injected with the glucocorticoid (GCD) triamcinolone acetonide (1.25 or 2.5 mg.100 g body wt-1.day-1 ip) for 2 days; they showed a profound increase in urinary excretion of Pi during the injection period. In clearance studies GCD increased the clearance and fractional excretion of Pi but did not change the filtered load of Pi. Initial "uphill" Na+-gradient (Nao+ greater than Nai+)-dependent uptake of 32Pi by luminal brush-border membrane (BBM) vesicles prepared from renal cortex of rats treated with GCD was markedly (greater than 40%) decreased compared with control rats; Na+-gradient-dependent uptake of D-[3H]glucose was not diminished. At the "equilibrium" time interval, measured at 120 min, BBM vesicles from control and GCD-treated rats did not differ in the uptake of 32Pi or D-[3H]glucose. With kinetic analysis, BBM from GCD-treated rats showed a marked decrease (-40%) in the maximum velocity (Vmax) of initial Na+-dependent 32Pi uptake, but the apparent affinity of the BBM transport system for Pi (apparent Km = 0.078 mM Pi) was not different from that of controls. Alkaline phosphatase specific activity was much lower (-40%) in BBM from GCD-treated rats compared with controls, but the activities of three other BBM enzymes (maltase, leucine aminopeptidase, and gamma-glutamyl transferase) were not different. The addition of triamcinolone to BBM in vitro had no effect on either Na+-dependent uptake of 32Pi or alkaline phosphatase activity. The rate of GNG from alpha-ketoglutarate was significantly increased in cortical slices from GCD-treated rats adapted to LPD. Also, the NAD+-to-NADH ratio was higher in the renal cortex of GCD-treated rats, although the total content of NAD [NAD+ + NADH] was not different from controls. Renal excretory, BBM, and metabolic changes elicited controls. Renal excretory, BBM, and metabolic changes elicited by GCD treatment were similar in intact and thyroparathyroidectomized rats. Phosphaturia elicited in rats fed LPD by GCD administration in vivo appears to be at least in part due to a decreased capacity of luminal BBM of proximal tubules for decreased capacity of luminal BBM of proximal tubules for Na+-dependent uptake of Pi. Although the causal relationship between observed parameters is not established, our results are compatible with the interpretation that an increase in the rate of renal GNG, perhaps via action of NAD+ on BBM (J. Clin. Invest. 67: 1347-1360, 1981), decreases luminal uptake and reabsorption of Pi in proximal tubules.
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PMID:Mechanism of glucocorticoid effect on renal transport of phosphate. 675 2

The effect of supplementing a cornsoybean diet (C) with glucose (G) or maltose (M) on young broilers (from hatch to 3 wk of age) affected by stunting syndrome (SS) was studied. Stunting syndrome was induced by orally administering an inoculum prepared from the intestines of SS broiler chicks. Relative to the M diet, the G diet improved growth and feed utilization and increased feed intake in naive (NA) control chickens. The C diet was intermediate in this respect. In contrast to the NA chickens, diet did not affect growth or feed utilization in SS chicks. Changes in the relative weights of the gastrointestinal tract segments were evident by 1 wk of age and hypertrophy of these segments persevered to 3 wk of age. Stunting syndrome infection was accompanied by a significant increase in pancreatic trypsin-specific activity during Weeks 1 and 2, and in chymotrypsin activity at 1 wk. During this time, amylase-specific activity was not affected. At 3 wk of age, the specific activities of amylase, trypsin, and chymotrypsin in the pancreas were lower in the inoculated vs control birds. Whereas no significant effect of SS was observed with activities of amylase in the intestinal contents, trypsin activity was higher in SS chicks at 1 wk, and that of chymotrypsin lower during Weeks 2 and 3. Relative to NA chicks, the maltase and saccharase activities of SS chicks were much lower during Week 1, but increased later on and were similar to NA chick values at 2 and 3 wk. Whereas the level of blood plasma proteins did not vary from 1 to 3 wk in the NA chicks, it increased gradually in SS chicks to a level that significantly exceeded that in their NA counterparts. Blood plasma glucose and triglyceride levels were slightly lower in the SS chicks (NS), and the blood plasma cholesterol level was significantly reduced during Week 2. Relative to NA chicks, SS infection caused a significant increase in plasma calcium during Weeks 2 and 3, accompanied by a significant reduction in blood plasma phosphorus at 2 wk only. No difference was observed in the blood plasma level of uric acid, which peaked in both treatments during Week 2, or in D-beta-hydroxybutyric acid level, which was quite stable during the experimental period. Stunting syndrome infection was accompanied by a dramatic increase in amylase and alkaline phosphatase activities in the blood plasma, and by a slight but significant decrease in activity of lactic dehydrogenase. Stunting syndrome was concluded to be an affliction not only of digestion but also of metabolism. The main depression in growth caused by SS inoculation is probably due to metabolic alterations beyond those of digestion and absorption.
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PMID:Stunting syndrome in broilers: effect of glucose or maltose supplementation on digestive organs, intestinal disaccharidases, and some blood metabolites. 905 21

We tested whether seasonal changes in the sources of organic substances for microbial metabolism were reflected changes in the activities of five extracellular enzymes in the eighth order lowland River Elbe, Germany. Leucine aminopeptidase showed the highest activities in the water column and the sediments, followed by phosphatase > beta-glucosidase > alpha-glucosidase > exo-1,4-beta-glucanase. Individual enzymes exhibited characteristic seasonal dynamics, as indicated by their relative contribution to cumulative enzyme activity. Leucine aminopeptidase was significantly more active in spring and summer. In contrast, the carbohydrate-degrading enzymes peaked in autumn, and beta-glucosidase activity peaked once again in winter. Thus, in sediments, the ratio of leucine aminopeptidase/beta-glucosidase reached significant higher medians in spring and summer (5-cm depth: ratio 7.7; 20-cm depth: ratio 10.1) than in autumn and winter (5-cm depth: ratio 3.7, 20-cm depth: ratio 6.3). The relative activity of phosphatase in the sediments was seasonally related to both the biomass of planktonic algae as well as to the high content of total particulate phosphorus in autumn and winter. Due to temporal shifts in organic matter supply and changes in the storage capacity of sediments, the seasonal peaks of enzyme activities in sediments exhibited a time lag of 2-3 months compared to that in the water column, along with a significant extension of peak width. Hence, our data show that the seasonal pattern of extracellular enzyme activities provides a sensitive approach to infer seasonal or temporary availability of organic matter in rivers from autochthonous and allochthonous sources. From the dynamics of individual enzyme activities, a consistent synoptic pattern of heterotrophic functioning in the studied river ecosystem could be derived. Our data support the revised riverine productivity model predicting that the metabolism of organic matter in high-order rivers is mainly fuelled by autochthonous production occurring in these reaches and riparian inputs.
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PMID:Regulation and seasonal dynamics of extracellular enzyme activities in the sediments of a large lowland river. 1620 47

Protein bodies and spherosomes isolated from mature seeds of Sorghum bicolor (Linn.) Moench have measurable activity of acid protease, alpha-glucosidase, beta-glucosidase, beta-galactosidase, phytase, acid pyrophosphatase, p-nitrophenyl phosphatase, and RNase. Protein bodies have largely insoluble activities, and produce soluble protein and soluble amino nitrogen during autolysis. They have the dual function of protein storage and protein catabolism. Spherosomes have considerable amounts of soluble enzymes and autolytically produce soluble amino nitrogen and inorganic phosphate but release little soluble protein. Spherosomes are similar to animal lysosomes but have an additional storage function for protein, phosphorus, and metals. Mature sorghum seed contains the necessary enzymes and substrates to generate two basic metabolites, amino acids and inorganic phosphate.
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PMID:Acid Hydrolases and Autolytic Properties of Protein Bodies and Spherosomes Isolated from Ungerminated Seeds of Sorghum bicolor (Linn.) Moench. 1665 31

To find out microbial metabolic functioning and toxicity in a former sawmill area, carbon dioxide evolution, methane oxidation potential, 10 hydrolytic enzyme activities, Vibrio fischeri test, fluorescein diacetate hydrolysis activity (FDA), soil pH, carbon, nitrogen and pentachlorophenol (PCP) content were measured at four sites. The area is contaminated with aged chlorophenols. Chlorophenol content of soil was analyzed with a novel HPLC-MS technique, which allowed to measure chlorophenols without derivatization. The sites had a pollution gradient from 0.5 to 15 microg PCP g dw of soil(-1). Endogenous carbon dioxide evolution, methane oxidation potential, butyrate-esterase, acetate-esterase, sulphatase and aminopeptidase activities were lower at the site 2 than 3, although the site 2 and 3 had similar content of carbon and nitrogen. The soil was toxic in V. fischeri test at the site 2, which had high content of PCP (3.93+/-1.00 microg PCP g dw of soil(-1)). The results indicated that endogenous carbon dioxide evolution, methane oxidation potential, butyrate-esterase, acetate-esterase, sulphatase and aminopeptidase activities were sensitive to PCP in the soil. The results indicated that alpha-glucosidase, beta-glucosidase, beta-xylosidase, beta-cellobiosidase, phosphomonoesterase, N-acetyl-glucosaminidase activity and FDA hydrolysis activity were not sensitive to PCP in the soil. Soil processes involved in the cycling of carbon, nitrogen, sulphur and phosphorus were only slightly vulnerable in the former sawmill area and most sensitive microbial species were probably replaced with more tolerant ones to maintain and recover functioning of the former sawmill soils.
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PMID:Microbial activities in soils of a former sawmill area. 1711 24

Pompe disease is a rare, progressive disease leading to skeletal muscle weakness due to deficiency of the acid alpha-glucosidase (GAA) enzyme. Herein we report the first diagnosed Finnish patient with a phenotype compatible with the late-onset form of Pompe disease. Molecular genetic analysis of the GAA gene revealed a novel missense mutation, 1725C>A (Y575X), combined with a previously reported mutation, 1634C>T (P545L). Human recombinant alpha-glucosidase enzyme (alglucosidase-alpha) treatment was initiated for this patient at age 20 years. After 12 months she was no longer fully wheelchair-bound, and muscle strength had improved. No disease progression was visible on muscle magnetic resonance imaging of the lower limbs, and the energy state of the muscle cells increased by 46% on phosphorus magnetic resonance spectroscopy. Overall, our findings suggest that enzyme replacement therapy is indicated, even in patients with late-onset Pompe disease, to halt disease progression and improve the quality of daily life.
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PMID:A novel mutation of the GAA gene in a Finnish late-onset Pompe disease patient: clinical phenotype and follow-up with enzyme replacement therapy. 1947 53


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