Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.23 (beta-galactosidase)
 14,648 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixteen pregnancies at risk for Gaucher disease -- six with the Norrbottnian form, one with a juvenile form with a similar clinical course to the patients from Norrbotten and nine with the infantile from -- have been monitored by the assay of beta-glucosidase activity in cultivated amniotic fluid cells with natural labelled glycosylceramide as substrate. Two methods of cultivation were compared in respect of their effect on the activity of lysosomal enzymes. No significant difference was found between the two marker enzymes, beta-galactosidase and N-acetyl-beta-glucosaminidase, but the beta-glucosidase activity was significantly higher in the cells cultivated with one of the methods. In four of the pregnancies at risk, the beta-glucosidase activity in the cultivated amniotic fluid cells was less than 5% of that in the two control materials. These fetuses were regarded as affected with Gaucher disease and were aborted. Differentiation between controls and Gaucher heterozygotes was not possible in cultivated amniotic fluid cells. The diagnosis of Gaucher disease in the amniotic fluid cells was confirmed in three of the four cases by the assay of the beta-glucosidase activity in the liver nd brain of the aborted fetuses. The glucosylceramide content of the liver from two aborted fetuses was not augmented. The beta-glucosidase activity was examined in seven placentas from pregnancies at risk for Gaucher disease and found to be in agreement with that in the cultivated amniotic fluid cells.
 ...
PMID:Prenatal diagnosis of Gaucher disease. Assay of the beta-glucosidase activity in amniotic fluid cells cultivated in two laboratories with different cultivation conditions. 678 Feb 54

Several glycosidase activities were measured in frontal gray matter of 4 brains from subjects affected by Creutzfeldt-Jakob disease. The changes of N-acetyl-beta-glucosaminidase, N-acetyl-beta-galactosaminidase, beta-glucosidase, alpha-fucosidase and alpha-mannosidase were not statistically significant but significant increases of beta-glucuronidase and beta-galactosidase activities were found. These results are in accordance with several reports on brain glycosidases in scrapie and Semliki Forest virus-infected brain and could explain some changes in brain glycoconjugate content previously observed in human and experimental Creutzfeldt-Jakob disease.
 ...
PMID:Brain glycosidases in Creutzfeldt-Jakob disease. 678 37

Although the cellular mechanisms controlling bile flow and biliary lipid secretion are unclear, morphologic data suggest that intracellular vesicles may be involved. Therefore, to investigate the role of hepatocyte lysosomes in bile flow and biliary lipid secretion, we studied the effect of cholestasis on biliary lipid output and on lysosomal enzyme activities and total protein concentration in liver and bile. Castrated male rats were treated with ethinyl estradiol at 0.5 or 5 mg/kg per day for 5 days; bile was collected through a complete bile fistula hourly for 4 hours, and then liver homogenates were prepared. Bile acids, cholesterol, and phospholipid were measured in bile, and three lysosomal glycosidases (beta-glucuronidase, beta-galactosidase, and N-acetyl-beta-glucosaminidase) and total protein were measured in bile and liver. Ethinyl estradiol inhibited bile flow in a dose-dependent fashion; it also inhibited bile acid and phospholipid outputs. In contrast, a marked and parallel increase in the biliary outputs of all three lysosomal hydrolases was observed after high-dose ethinyl estradiol; no change in the biliary concentration of total protein was found. Our data suggest that bile flow and biliary lipid secretion involve cellular mechanisms other than vesicular transport by lysosomes.
 ...
PMID:Dissociation of bile flow and biliary lipid secretion from biliary lysosomal enzyme output in experimental cholestasis. 678 57

Autolysis of a homogenate from the human gastric mucosa results in the release of the reducing substances into the supernatant. The activities of the six glycosidases from the human gastric mucosa, N-acetyl-beta-glucosaminidase, N-acetyl-Beta-galactosaminidase, alpha-fucosidase, beta-galactosidase, alpha-mannosidase and alpha-glucosidase were determined. Certain properties of the enzymes are described. The significance of these enzymes in glycoprotein catabolism in the human gastric mucosa is discussed.
 ...
PMID:The degradation of glycoconjugates in the human gastric mucous membrane. 679 8

Histochemical techniques have been used to study the chemical composition of the oesophageal gland secretions of Orthocoelium scoliocoelium and Paramphistomum cervi. Results suggest that the secretions contain numerous enzymes, e.g., non-specific alkaline and acid phosphatases, ATPase, TPPase, esterase (Cathepsin C like), beta-galactosidase, beta-glucuronidase and N-acetyl-beta-glucosaminidase. The role of these enzymes in the digestion of food in these amphistomes is discussed. On the basis of histological and histochemical studies the caecal epithelium of the two amphistomes has been found to be syncytial bearing regularly arranged numerous, long but equal-sized and closely packed cylindrical microvilli. The role of various hydrolytic enzymes in well fed and starved flukes in relation to their gastrodermis and microvilli has also been discussed.
 ...
PMID:Role of oesophageal glands in the digestive physiology of two rumen amphistomes Orthocoelium scoliocoelium and Paramphistomum cervi. 684 57

The biochemical activities of 8 lysosomal acid hydrolases in leukemic cells from 48 patients were examined. Characteristic alterations were found in alpha-mannosidase, beta-galactosidase and N-acetyl-beta-glucosaminidase activities of leukemic cells. The level of alpha-mannosidase activity was much higher in myelo(mono)genous leukemias (AML, AMoL, AMMoL, CML and CMMoL) than in lymphogenous ones (ALL, T-cell leukemia, hairy cell leukemia and CLL) without exception. The beta-galactosidase activity also differed as a result of alpha-mannosidase, except in T-cell leukemia. In T-cell leukemia it was within the range of normal lymphocytes, but in the other lymphogenous leukemias it was significantly below normal. N-acetyl-beta-glucosaminidase activity in myelo(mono)genous leukemic cells was above the range of normal granulocytes. The changes in these enzyme levels were consistent. The lymphocytic or myelocytic nature of three cases of acute undifferentiated leukemia could be determined by enzyme studies. In two cases it was lymphocytic and in one it was myelocytic. The enzymatic abnormalities were also found in morphologically mature neutrophils from patients but not only chronic types (CML, CMMoL) but also acute types (AMoL, AMMoL) of leukemias, and were similar to those of their respective leukemic cells. Analysis of lysosomal enzymes (at least three of those mentioned above), can elucidate one of the biochemical properties of leukemic cells and may be valuable in the differentiation of leukemias.
 ...
PMID:Biochemical activities of lysosomal acid hydrolases in leukemic cells. 694 81

The API ZYM system (Analytab Products, Plainview, N.Y.), containing 19 chromogenic substrates, was utilized semiquantitatively to detect extracellular acid and alkaline phosphatases, aminopeptidases, proteases, esterase-lipase, phosphoamidase, and glycosidases in 128 oral and nonoral isolates of black-pigmented Bacteroides, Actinobacillus, Haemophilus aphrophilus, Capnocytophaga, Fusobacterium nucleatum, Wolinella recta, and Veillonella parvula. In the black-pigmented Bacteroides group of organisms, a strong trypsin reaction was present in Bacteroides gingivalis (oral species) but not in Bacteroides asaccharolyticus (nonoral species). Bacteroides melaninogenicus subsp. melaninogenicus, in contrast to Bacteroides melaninogenicus subsp. intermedius, exhibited strong N-acetyl-beta-glucosaminidase activity. H. aphrophilus produced beta-galactosidase and alpha-glucosidase, but the closely related Actinobacillus actinomycetemcomitans did not. Capnocytophaga was distinct with respect to strong aminopeptidase reactions. This study showed that a wide range of enzymes which have the potential of causing tissue injury and inflammation can be elaborated from major oral gram-negative species. Also, the API ZYM system appears to be a valuable adjunct to traditional biochemical testing in identifying oral gram-negative species.
 ...
PMID:Enzymatic characterization of some oral and nonoral gram-negative bacteria with the API ZYM system. 702 98

Thirty strains of Propionibacterium acnes were grown in basal salt medium containing lecithin as a lipid substrate and in other media. The cultures were assayed for production of lipase (measured as fatty acid esterase) and other exoenzymes. Lipase was assayed spectrophotometrically; other enzymes were assayed using the API ZYM system (Analytab Products Inc., Plainview, NY). Substance for lipase were alpha- and beta-naphthol esters of propionic, butyric, valeric, caprylic, lauric, myristic, and oleic acids. All strains showed fatty acid esterase activity. Using the API ZYM system 19 enzymes were detected, 8 of which were found frequently and had high activity in most strains. Acid and alkaline phosphatases, phosphoamidase, ester lipase, trypsin-chymotrypsin-like proteases, beta-glucuronidase (80%), beta-galactosidase (80%), and N-acetyl-beta-glucosaminidase were found. Many enzymes of P. acnes appear to be adaptive, dependent on the culture substrate.
 ...
PMID:Exoenzymes of Propionibacterium acnes. 717 37

The effect of CH-123 (3-carbethoxy-6-methyl-1-9-(carboxy-methyl)-1-4-oxo-6,7,8,9-tetrahydro-4H-pyrid o(1,2a)pyrimidine) was investigated on the activity of 4 lysosomal enzymes: beta-glucuronidase, beta-galactosidase, N-acetyl-beta-glucosaminidase and acid phosphatase obtained from aortic smooth muscle and liver cells of rabbits. Animals were fed on a 2% cholesterol diet for 4 weeks and used an experimental atherosclerotic group. In drug-treated groups, after 4 weeks of cholesterol feeding the diet was changed to regular food and the animals were treated daily either with 50 mg/kg CH-123 or with 250 mg/kg Clofibrate. The postnuclear supernatant of homogenates of liver and aortic cells was isolated, lysosomes were fractionated by sucrose density gradient centrifugation, and the activity of enzymes was measured. In cholesterol-fed animals the enzyme activities of aorta and liver was 3-5 times higher than in the control, i.e. in the group of rabbits fed regular food. On Clofibrate treatment the enzyme activities were 2-3 times higher, but on treatment with CH-123, they were only 1.2-1.8 times above the control. Experiments suggest that CH-123 treatment suppresses the elevated lysosomal marker enzyme activities in aortic and liver cells of atherosclerotic animals.
 ...
PMID:Effect of CH-1243, a pyrido (1,2-a) pyrimidine derivative on the elevated activity of lysosomal enzymes of rabbit aorta and liver in experimental atherosclerosis. 724 98

Two forms of alkaline phosphatase (orthophosphoric monoester phosphohydrolase (alkaline optimum, EC 3.1.3.1) have been purified from human small intestine by column chromatography on DEAE-cellulose and tyraminyl derivative affinity gel, and by preparative disc gel electrophoresis. Intestinal phosphatases were electrophoretically separated into two components, fast- and slow-moving enzymes, with apparent molecular weights of 140000 and 168000 and with subunit weights of 68000 and 80000, respectively. Analyses of carbohydrate and amino acid revealed marked differences in the two enzymes. Enzymatic properties and affinities for an anti-blood group antibody were also found to differ. Papain digestion released a hydrophobic small peptide from the slow-moving enzyme and its enzymatic properties resembled those of the fast-moving enzyme. Circulating clearance (T1/2) of the slow- and fast-moving enzymes from adult intestine was found to be 7.5 h and 1.3 h, respectively; that of fetal intestinal enzyme was 2.8 h. Sialidase, sialidase/beta-galactosidase, or sialidase/beta-galactosidase/N-acetyl-beta-glucosaminidase treatment of the fetal enzyme reduced the value to about 40 min. Further, digestion with alpha-fucosidase, alpha-mannosidase or both restored it to nearly the original level. Organ distribution of injected 125I-labelled enzymes indicates that the desialylated hepatic enzyme was selectively distributed in liver, while the degalactosylated intestinal enzyme was incorporated into liver lymph fluid, and small intestine. These results suggest that the pathway of circulating clearance of alkaline phosphatase has several routes.
 ...
PMID:Multiple forms of human intestinal alkaline phosphatase: chemical and enzymatic properties, and circulating clearances of the fast- and slow-moving enzymes. 730 75


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>