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

Urinary excretion of lactate dehydrogenase, hydroxybutyrate dehydrogenase, gamma-glutamyltransferase, alkaline phosphatase, arylsulphatase A, alpha-glucosidase, beta-galactosidase, trehalase, N-acetyl-beta-glucosaminidase, beta-glucuronidase, and leucinearylamidase was studies in a carefully selected group of 100 healthy subjects, 50 women and 50 men. Enzyme activities were assayed in 3-h morning samples after gel filtration of the urine. Activities were related to time volume, and to urinary creatinine concentration. Several transforming functions had to be applied to enzyme output data to obtain an approximation to gaussian frequency distribution. Men showed a significantly higher excretion of gamma-glutamyltransferase, alpha-glucosidase, trehalase, N-acetyl-beta-glucosaminidase,beta-glucuronidase, and leucine arylamidase activity than did women if enzyme activity was related to urinary time volume. Women excreted more lactate dehydrogenase, hydroxybutyrate dehydrogenase, gamma-glutamyltransferase, alkaline phosphatase, alpha-glucosidase, trehalase, and N-acetyl-beta-glucosaminidase activity than did men, if urinary creatinine was used as the basis of reference. Reference intervals were calculated as 2.5 and 97.5 percentiles for both sexes.
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PMID:Normal limits of urinary excretion of eleven enzymes. 1 92

Variations in the urinary excretion of arylsulphatase A, beta-galactosidase, alpha-glucosidase and beta-glucuronidase throughout a 24-h period were studied in 8 healthy subjects. Urine was collected at 3-h intervals and enzyme activities were assayed after gelfiltration of the urine specimens. Significant intra-individual changes of the excretion of all 4 enzymes during the 24-h period were found. Enzyme output was high between 3 a.m. and 9 a.m. and low during the afternoon and evening hours. The most striking pattern was seen for arylsulphatase A. Diurnal variations of urinary enzyme excretion seemed not to be flow dependent. Both modes of expression of enzyme output (mU/min or U/g creatinine) gave corresponding results. It is concluded that for the measurement of the excretion of these enzymes urine should be collected during a fixed time interval, e.g. from 6 a.m. to 9 a.m.
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PMID:Diurnal variations of urinary enzyme excretion. 1 45

Assay conditions have been developed for the determination of urinary beta-glucuronidase, beta-galactosidase, alpha-galactosidase, and beta-hexosaminidase using fluorometric substrates. The assay conditions for beta-glucuronidase overcome interference by both low and high molecular weight inhibitors, a problem that has confused earlier studies of enzyme excretion. The four lysosomal enzymes are excreted corrdinately: although their absolute levels (in units per milligram of creatinine) vary during the day and from one day to the next, the ratio of one enzyme to another remains relatively constant. The lack of correlation betweem plasma and urine enzyme levels, together with the high molecular weights of these enzymes, suggests that the urinary enzymes are not derived by glomerular filtration. The lack of coordinacy with lactate dehydrogenase suggests they are not derived from exfoliated cells. by analogy with experimental animals, they may be derived from lysosomes extruded into the lumen of the proximal tubule by epithelial cells. There is considerable variation among a population of 125 healthy adult subjects for total enzyme excretion. Both total enzyme excretion and coordinacy ratios are log-normally distributed, suggesting that they are the resultants of many factors, each of which has a relative, or proportional, effect on enzyme excretion. About one-half the population variation resides in a process common to the excretion of all four enzymes (possibly the lysosome extrusion pathway), and about one-half resides in factors affecting each enzyme independently.
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PMID:Coordinacy of lysosomal enzyme excretion in human urine. 2 85

Three urinary lysosomal enzymes, beta-glucuronidase (beta-Gluc), beta-galactosidase (beta-Gal) and N-acetyl-beta-D-glucosaminidase (NAG), were measured in twenty-one renal allograft recipients to evaluate their role in the diagnosis and prediction of rejection episodes, and in the prediction of eventual graft outcome. A fluorometric assay using methylumbelliferone substrates was used to measure the three enzymes in morning urine samples and enzyme activity was defined in terms of urine creatinine concentration. Urinary NAG levels increased significantly in 13/16 first rejection episodes and 4/4 instances of acute tubular necrosis and graft infarction. In 5 of the 16 first rejection episodes the NAG was predictive of the rejection. NAG was not useful in diagnosing second or subsequent rejections and beta-Gluc and beta-Gal were of little value in assessing any component of renal transplant pathology. As a prognostic index of eventual graft outcome, the peak urinary NAG was particularly encouraging. It correlated strongly with deterioration in graft function as time passed such that only 2/10 patients with peak NAG greater than 1400 Units had normal serum creatinines at 6 months post transplantation. Conversely 4/4 patients with peak NAG levels less than 700 Units had normal serum creatinine at that time. In our series the measurement of urinary NAG was a useful adjunct to the diagnosis of first rejections but appears to be more valuable in predicting graft outcome.
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PMID:Urinary lysosomal enzyme excretion after renal allotransplantation. 10 10

The proteinuria rate and the relative clearances of beta 2-microglobulin, orosomucoid, albumin, transferrin and IgG were measured in forty-two workers exposed to cadmium and in seventy-seven control workers. A tubular type proteinuria with an increased excretion of beta 2-microglobulin and often also a glomerular type proteinuria with an increased excretion of orosomucoid, albumin, transferrin and IgG were observed mainly in workers exposed to cadmium for more than 25 years and whose cadmium concentration in blood exceeded 1 microgram Cd/100 ml and that in urine 10 microgram Cd/g creatinine. The glomerular dysfunction was also suggested by an increased plasma level of beta 2-microglobulin and creatinine. Both tubular and glomerular impairments occurred with the same prevalence and were not necessarily associated. The increased release of beta-galactosidase by the kidney suggested that cadmium can damage some epithelial cells.
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PMID:Renal excretion of proteins and enzymes in workers exposed to cadmium. 11 May 96

Plasma and urine beta-N-acetyl glucosaminidase, beta-glucuronidase and beta-galactosidase were measured in 75 diabetics and 35 control subjects. The plasma enzyme levels were significantly elevated in patients with evidence of vascular complications. There was a negative correlation between plasma enzymes and creatinine clearance.
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PMID:Serum and urine glycosidase activities in diabetes mellitus. 11 88

A method is described for the detection of abnormal oligosaccharides in a small (5 ml) volume of urine, employing filtration on a Bio Gel P-6 column, determination of neutral sugar and bound sialic acid, and determination of creatinine content. With this method increased urinary excretion of sialic acid-rich oligosaccharides has been detected in nine patients with mucolipidoses (five cases of mucolipidosis II and four patients of mucolipidosis, with beta-galactosidase deficiency). The filtration patterns of oligosaccharides in mucolipidoses were clearly distinguishable from those in other inborn errors of metabolism. Total excreted oligosaccharides were increased 5--30-fold in these patients; mucolipidosis II, 640--1350 microgram neutral sugar/mg creatinine; control 54 +/- 20 microgram neutral sugar/mg creatinine. The oligosaccharides consisted of three sialic acid-rich fractions and were common in both types of mucolipidosis. Our data indicate that hypersialyoligosacchariduria is the main biochemical feature of both types of mucolipidosis.
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PMID:Hypersialyloligosacchariduria in mucolipidoses: a method for diagnosis. 65 39

Six patients who received renal transplants were closely monitored to compare the sensitivity of urine levels of beta-galactosidase and N-acetyl-beta-glucosaminidase with conventional clinical and laboratory parameters in the detection of impending rejection. A rapid (60 minute), simple, accurate fluorometric assay was used to measure activities of both enzymes. Eighty per cent of ten rejection episodes were accompanied by a two- to sixfold increase in enzyme release. Parallel changes in serum creatinine levels and urinary volume occurred in six rejection episodes, but in two episodes, elevated urinary enzyme levels were observed two and four days prior to clinical evidence of rejection. It is concluded that urinary lysosomal enzyme measurements by fluorometric assay are valuable indicators of acute renal rejection, particularly when the diagnosis is not clearly established by conventional criteria that show only minimal changes. Continuing studies in a large group of renal transplant recipients are under way to evaluate the validity of this conclusion and to determine whether enzyme measurements, will, indeed, be indicative of early rejection.
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PMID:Urinary lysosomal enzymes for detection of acute renal allograft rejection. 77 11

The activities of four lysosomal enzymes and creatinine levels were measured in the plasma and urine of 17 healthy elderly and 7 young adults. Fractional enzyme excretion (FE ENZ) values for beta-hexosaminidase (N-acetylglucosaminidase), alpha-galactosidase, beta-galactosidase and beta-glucuronidase were calculated and compared between the two groups of subjects. FE ENZ was calculated as the ratio of enzyme clearance to creatinine clearance. The FE ENZ values for alpha-galactosidase, beta-galactosidase and beta-glucuronidase between the elderly and young populations were not statistically different; however, relative to the young control group, the FE ENZ value for beta-hexosaminidase was elevated approximately 2-fold in the elderly population (P = 0.06). The mean urinary alpha-galactosidase activity for the elderly population, when expressed on the basis of creatinine, was 50% lower than that of the control group (P = 0.03), whereas the mean urinary beta-hexosaminidase activity for the elderly was significantly higher compared to the control group (P = 0.008). When data for all subjects was analyzed, no correlation was observed between the urinary excretion of beta-hexosaminidase or alpha-galactosidase and glomerular filtration rate. These data indicate that with advancing age there are changes in the tubular secretion or reabsorption of selective lysosomal enzymes, particularly beta-hexosaminidase and alpha-galactosidase. These biochemical changes may provide a means of assessing subtle progressive deterioration of renal function.
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PMID:Comparison of urinary excretion of four lysosomal hydrolases in healthy elderly and young adults. 133 Mar 76

Urinary dilution adjustment methods can be used to reduce the intra-individual variability in concentrations of metals and other substances in urine due to variability in urinary flow. In this study linear and non-linear dilution adjustments with urinary flow, creatinine (CREAT) and urinary density (UD) were compared for the urinary enzymes alanine amino peptidase (AAP), beta-galactosidase (beta GAL) and N-acetyl-beta, D-glucosaminidase (NAG). The most optimal dilution adjustment for AAP was: AAPadjusted = AAPmeasured/(CREATmeasured)0.824 The optimal dilution adjustment for beta GAL was: beta GALadjusted = beta GALmeasured/(CREATmeasured)0.878 For NAG the optimal dilution adjustment parameter was the conventional linear adjustment with SG. It could not be determined whether urinary dilution methods can be useful for population based reference intervals of urinary enzymes. If personal reference intervals can be calculated, urinary dilution adjustment methods may be useful by reduction of intraindividual variability.
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PMID:A comparison of dilution adjustment methods for urinary enzymes. 135 36


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