Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.2.1.31 (beta-glucuronidase)
7,680 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The ability of epsilon-amino caproic acid (EACA)-treated normal serum and of cystic fibrosis (CF)-affected and carrier sera to promote the release of lysosomal enzymes from sensitized human polymorphonuclear leukocytes (PMN) was assessed through the measurement of beta-glucuronidase and myeloperoxidase activity after exposure of these cells to the various test sera. This study was initiated to extend the analogies between preciliary dyskinesia factor (pre-CDF), separated from the cell-free media of cultures derived from CF homozygous and heterozygous individuals, and C3a anaphylatoxin. The extent of lysosomal degranulation of human PMN exposed to fresh untreated sera of each of five controls, seven CF homozygotes, and eight heterozygotes, as expressed by the amount of beta-glucuronidase releases, was 7.84% (+/- 0.934) for countrol sera, 14.01% (+/- 1.79) for CF-affected sera, and 10.61% (+/- 1.43) for heterozygous sera. The difference between CF homozygotes and control subjects is significatn (P less than 0.0001), as is the difference between CF-affected and carrier individuals (0.001 less than P less than 0.005) and between control subjects and carriers (0.001 less than P less than 0.005), when beta-glucuronidase. However, the differences between control subjects and CF heterozygous individuals are not significant. Treatment of these sera with 1 M EACA gave values for beta-glucuronidase and myeloperoxidase release which are slightly reduced when compared with those obtained with fresh, untreated samples. EACA apparently reduces the activity of beta-glucuronidase released from PMN. Amicon filtration studies of these serum samples demonstrated that degranulating ability and the presence of cilicary dyskinesia, as assessed by rabbit tracheal bioassay, are not always associated. Therefore, the relationship between pre-CDF and the degranulator activity in native CF-affected and carrier sera is unclear, in part because of the limitations inherent in the test systems employed.
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PMID:Demonstration of human leukocyte degranulation induced by sera from homozygotes and heterozygotes for cystic fibrosis. 17 51

The specific activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase were studied in meconium from infants with cystic fibrosis (CF) and control subjects. The study revealed significant variations in the specific activity of the enzymes except for acid phosphatase. The variations were not uniform. The activities of alpha-D-mannosidase, beta-glucuronidase and alkaline phosphatase were markedly decreased (p less than 0.001, p less than 0.002, p less than 0.001, respectively), while the activity of beta-D-fucosidase was significantly increased (p less than 0.001) in meconium from the infants with CF. It is suggested that the decreased activity of alpha-D-mannosidase and beta-glucuronidase might contribute to the accumulation of the abnormal substances in CF meconium. The highly increased activity of beta-D-fucosidase raises the possibility of an additional or alternative method for screening newborns for CF using meconium as the test material.
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PMID:Studies in meconium in cystic fibrosis: the activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase. 21 31

The presence in the serum of both cystic fibrosis (CF) homozygotes and heterozygotes of a factor inhibiting the response of lymphocyte lysosome beta-glucuronidase activity to in vitro phytohaemagglutinin (PHA) stimulation is confirmed. Studying lymphocyte beta-glucuronidase activity on PHA stimulation represents a way to confirm CF diagnosis and to screen CF heterozygotes. For technical complexity, however, the method cannot be used for mass screening, but it can be useful for confirming the diagnosis in suspected cases. Relationships between serum factor inhibiting the effect of PHA on beta-glucuronidase, ciliary dyskinesia factor and carboxypeptidase B-like activity are discussed.
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PMID:Further studies on lymphocyte beta-glucuronidase abnormality in cystic fibrosis. Possible relations to carboxypeptidase B-like activity. 73 2

Lymphocytes from adults homozygous or heterozygous for cystic fibrosis show biochemical abnormalities when cultured for 48 hours in the presence of phytohemagglutinin and autologous serum. In contrast to the 45 per cent increase in total protein and beta-glucuronidase concentrations seen in healthy control subjects when measured per 10(10) cells, both concentrations decreased by 1 per cent in adults heterozygous for cystic fibrosis and by 18 per cent in adults homozygous for cystic fibrosis. The abnormal response of the lymphocytes from persons with cystic fibrosis was due to a serum factor and not to any intrinsic abnormality of the lymphocytes. An abnormal response to hytohemagglutinin occurred in only 14 per cent of 44 healthy control subjects, but in 100 per cent of 14 adults homozygous for cystic fibrosis and in 85 per cent of 26 adults presumed to be heterozygous for cystic fibrosis. As a result of this phenomenon, lymphocytic beta-glucuronidase concentrations were significantly lower than normal in patients with cystic fibrosis when the cells were cultured with phytohemagglutinin and autologous serum. The demonstration of this phenomenon in both homozygotes and presumed heterozygotes (parents) suggests a relationship to the genetic defect in cystic fibrosis.
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PMID:Abnormal response of cultured lymphocytes to phytohemagglutinin and autologous serum in cystic fibrosis. 93 Nov 81

The regulation of mucin secretion by SW1116 human colon carcinoma cells has been studied using monoclonal antibody 19-9, which has previously been used to detect mucin in the serum of cancer and cystic fibrosis patients. We found that SW1116 cells constitutively secrete considerable amounts of mucin as the predominant glycoprotein. The secretion of mucin by these cells is independent of cyclic AMP levels, but can be further stimulated by the Ca2+ ionophore A23187. However, arachidonic acid and its metabolites inhibit mucin secretion. Electron microscope studies reveal that the mucin is located near the plasma membrane as well as in vesicular and lysosome-like structures. However, the secretion pathway of mucin is different than that of the lysosomal contents, since arachidonic acid, while inhibiting mucin secretion, actually activates the secretion of the lysosomal enzyme beta-glucuronidase. We suggest that the mechanism of mucin secretion by SW1116 cells occurs by a pathway different from common exocytosis, and possibly by more than one pathway. The response of mucin secretion by SW1116 cells to common secretagogues resembles that of epithelial cells obtained from cystic fibrosis patients. Thus SW1116 cells are an especially interesting system for studying processes related to pathological states associated with excessive constitutive secretion of mucin.
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PMID:Cyclic AMP-independent secretion of mucin by SW1116 human colon carcinoma cells. Differential control by Ca2+ ionophore A23187 and arachidonic acid. 137 31

Neutrophils from cystic fibrosis (CF) patients have been shown previously to be defective in their response (beta-glucuronidase exocytosis, NADPH oxidase activation) to the chemotactic peptide FMLP. In this work, we attempted to identify the defective step in this response. We showed that stimulated CF and control neutrophils do not differ in the formation of inositol phosphates. On the other hand, direct stimulation of protein kinase C with phorbol myristate acetate (PMA) revealed a subnormal stimulation of beta-glucuronidase exocytosis in CF neutrophils. Furthermore, retroinhibition exerted by PMA-activated protein kinase C on stimulated inositol phosphates or on beta-glucuronidase exocytosis was marginal or absent in CF neutrophils, whereas it was significant in the case of control neutrophils. Our observations suggest that the CFTR gene is expressed in neutrophils and is involved in protein kinase C-mediated actions.
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PMID:Defective protein kinase C-mediated actions in cystic fibrosis neutrophils. 189 35

In order to determine whether cystic fibrosis neutrophils are affected in their secretory functions, lysosomal enzyme release and chemiluminescence (light emission from cells) were assayed in patients' cells and compared with those in normal control cells. We observed a decreased response of cystic fibrosis neutrophils in beta-glucuronidase release and chemiluminescence after stimulation by N-formyl-methionyl-leucyl-phenylalanine. There was no significant correlation of these results with the clinical score nor with the medical treatment. On the other hand, responses to the calcium ionophore A23187 and to opsonized zymosan showed no significant difference between normal and cystic fibrosis subjects in lysosomal enzyme release. N-formyl-methionyl-leucyl-phenylalanine receptor alterations did not seem involved in the observed effect as demonstrated by Scatchard plot analysis of N-formyl-methionyl-leucyl-phenylalanine binding to these receptors. These results clearly demonstrate a difference between normal and cystic fibrosis neutrophils in release and chemiluminescence responses to N-formyl-methionyl-leucyl-phenylalanine stimulation, a difference that might be located in the plasma membrane as both responses are membrane dependent.
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PMID:Alteration of the N-formyl-methionyl-leucyl-phenylalanine-induced response in cystic fibrosis neutrophils. 308 28

The activities of a number of lysosomal hydrolases were determined in sera from 100 patients with cystic fibrosis (CF), age 2-35 yr, and age-matched controls: beta-hexosaminidase activity was significantly elevated (p less than 0.005) in CF patients from all age groups. alpha-Mannosidase activity was increased only in the older CF patients (greater than 13 yr). The following enzyme activities were not altered in CF serum: alpha-fucosidase, beta-glucuronidase and acid phosphatase. The abnormal patterns of serum alpha-mannosidase and beta-hexosaminidase in CF cannot be explained by pancreatic disease or undernutrition, since serum values of these hydrolases in patients with anorexia nervosa or acute pancreatitis were not altered. However, the altered activities of the alpha-mannosidase and beta-hexosaminidase were proportional to the degree of pulmonary insufficiency in the CF group, indicating that these changes are probably a secondary consequence of the primary disease process. Except for beta-hexosaminidase, because differences in the serum hydrolases in CF do not become apparent until the second decade of life, determinations of lysosomal enzyme activities in serum will probably be of little diagnostic value.
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PMID:Serum lysosomal hydrolases in cystic fibrosis. 316 78

We report on a patient with a de novo chromosome abnormality del(7)(q21.1q22). The cells of this patient were used to determine the assignment of the gene for the enzyme beta-glucuronidase and the DNA probes around the cystic fibrosis gene--pJ3.11 and metH. Both the beta-glucuronidase gene and the DNA probes pJ3.11 and metH were found in 2 copies in our patient, indicating that neither locus lies in the deleted segment.
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PMID:Interstitial 7q deletion [46,XX,del(7)(pter----q21.1::q22----qter)] and the location of genes for beta-glucuronidase and cystic fibrosis. 322 44

We measured the activity of a non-lysosomal alpha-glucosidase with pH optimum near 6.0 in serum from a wide variety of patients, using the fluorogenic substrate, 4-methylumbelliferyl-alpha-D-glucopyranoside. Acutely ill patients with cystic fibrosis (CF) demonstrated significant increases in alpha-glucosidase compared with CF outpatients. The former group of CF patients experienced far more severe chronic pulmonary disease than did the latter, whereas both groups had similar degrees of gastrointestinal impairment. Patients with pancreatitis associated with trauma or complicated by severe necrosis, hemorrhage, or abscess also displayed greater increases in alpha-glucosidase than did patients with uncomplicated (edematous) pancreatitis. For CF outpatients and patients with either edematous pancreatitis or pancreatic cancer, the alpha-glucosidase activity was similar to that for the general hospital-patient population. Corresponding changes were not observed for other measured serum glycosidases (alpha-fucosidase, alpha-mannosidase, beta-glucuronidase, beta-N-acetylglucosaminidase). Measurement of serum alpha-glucosidase may be of value in assessing the clinical course in CF and in differentiating necrotizing from edematous pancreatitis.
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PMID:Measurement of alpha-glucosidase activity in serum from patients with cystic fibrosis or pancreatitis. 351 92


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