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 purpose of this study was to compare the latencies of lysosomal enzymes (beta-glucuronidase, beta-N-acetylglucosaminidase, arylsulphatase and acid ribonuclease) in heart and in red and white skeletal muscle of male and female mice (Mus musculus). The unsedimentable, free activities together with releasable (Triton X-100, hypotonic shock and freeze-thawing treatments) and unreleasable, bound activities were assayed. The distribution of acid hydrolases to different fractions was strikingly heterogeneous. The most distinct differences occurred between the distributions of beta-glucuronidase and beta-N-acetylglucosaminidase. The differences between muscle types occurred in the activity levels of lysosomal enzymes, rather than in the fractional distributions. Sex-related differences were small and occurred mainly in the activity levels of heart muscle (higher in female mice). The results suggest that the heterogeneous distribution of lysosomal enzymes originates in the compartmental differences of lysosomal enzymes in muscle cells, rather than the differences in cell populations of different muscle types.
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PMID:Latency differences of lysosomal enzymes in cardiac and skeletal muscles of male and female mice. 286 81

We have examined the reversibility of the biochemical and pathological changes induced in the spleen, kidney and lung of the suramin-treated rat which we have previously proposed as a useful model of the human condition, mucopolysaccharidosis (MPS). Rats were injected with a single intravenous dose of suramin (250 mg/kg) and allowed to survive for periods of up to 6 months. The organs were examined for suramin content, pathological changes, biochemical storage of glycosaminoglycans (GAGs) and for the blockage of the relevant hydrolytic enzymes. The extent and rate of suramin accumulation and the retention of the drug varied considerably between organs with the greatest concentration of suramin (4,000 micrograms/g) occurring in the kidney 2 weeks after injection. Suramin persisted at gradually decreasing levels in all organs for the duration of the experiment, remaining at the highest level (1,150 micrograms/g) in the kidney. The concentration of GAGs peaked 10-18 days after administration of the drug, in all organs. Within 6 months the level had returned to normal in the liver, spleen and lung, but remained elevated in the kidney. The activities of beta-glucuronidase and acid phosphatase were decreased in all organs at diminishing levels throughout the experiment. There was a significant increase in the activity of arylsulphatase B, except in the kidney, where the predominant effect was a reduction of activity. Recovery from the morphological changes was evident in all organs except the lung within 6 months of suramin administration. The reversibility of the biochemical and pathological changes in the various tissues is discussed and compared with the earlier results described for the liver (Rees et al. 1986) and the implications of using suramin for the treatment of human trypanosomiasis, onchocerciasis and AIDS are considered.
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PMID:The suramin-treated rat as a model of mucopolysaccharidosis. Variation in the reversibility of biochemical and morphological changes among different organs. 287 81

Lysosomal changes were recorded in the skeletal muscles of mice and rats during the repair of muscle fiber injuries caused by a single bout of prolonged running. One purpose of the study was to characterize cellular and compartmental distributions of lysosomal enzymes and to investigate ultrastructural changes in the lysosomal system associated with the appearance and repair of muscle fiber injuries. Furthermore, the level of muscle fiber injuries was correlated with the lysosomal enzyme response and the indices obtained were utilized in the evaluation of the pathogenesis of exercise myopathy. The main results were: Heavy physical exertion caused scattered necrotic injuries of muscle fibers and inflammation in several skeletal muscles. The most susceptible muscles to exercise injuries were the red deep parts of quadriceps femoris muscle and the soleus and tibialis anterior muscles. The total activities of several lysosomal acid hydrolases, in particular those of beta/-glucuronidase, cathepsin C and arylsulphatase, strongly increased during the repair of exercise injuries. The highest responses occurred 3-5 days after exertion and the degree of enzyme responses correlated significantly with the level of histological injuries. The histochemical staining intensities of beta-N-acetylglucosaminidase and beta-glucuronidase were highest 3 and 5 days after exertion. An increased staining intensity occurred in the inflammatory phagocytes and in the surviving muscle fibers, especially in the red muscle fibers close to necrotic foci. An autophagic response occurred in the muscle fibers close to necrotic foci 2-7 days after exertion. Autophagic vacuoles were frequently small and contained different cellular structures at various stages of degradation. The number of lysosome-like bodies, Golgi complexes and multilamellar bodies also increased. Macrophages removed the debris of necrotic muscle fibers by heterophagic uptake 2-5 days after exertion. The unsedimentable and releasable activities of acid hydrolases increased more prominently than those of the total activities in homogenates and reached their highest values 3 days after exertion. The proportional distribution of various acid hydrolases to unsedimentable, releasable and bound fractions varied strikingly but remained appreciably stable throughout the exercise myopathy. The content of lysosomal phosphomannosyl-enzyme receptors in the membrane fraction was unchanged 0-3 days after exertion but a small increase occurred later. The endogenous receptor-bound activity of beta-N-acetylglucosaminidase was considerably increased 1-5 days after exertion but decreased later to the control level.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Lysosomal changes in skeletal muscles during the repair of exercise injuries in muscle fibers. 298 70

The influence of culture conditions on the ultrastructure and enzyme activities of amniotic fluid cells are reported. Morphological changes were determined as a function of the number of lysosomal-like inclusion bodies per cell, and these results correlated to the activity of beta-hexosaminidase, alpha-mannosidase, beta-glucuronidase, arylsulphatase C and 5' nucleotidase. The parameters examined were pH of the culture media, type of media, increasing cell passage and day of harvest. Our results indicate that enzyme activities are less sensitive to changes in culture conditions as compared to ultrastructural changes. We therefore recommend that in order to obtain reliable ultrastructural results for the diagnosis of storage disorders, cultures should be grown in MEM as the culture medium, the pH of the medium carefully monitored to remain below pH 7.4, examining the cultures no later than the eighth cell passage and no later than the 10th day after subculture.
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PMID:Cultured amniotic fluid cells for prenatal diagnosis of lysosomal storage disorders: a methodological study. 302 78

A 2.5-year-old girl who presented with abdominal distension, hepatomegaly, coarse facies, hirsutism and contraction deformities was investigated for mucopolysaccharidoses. Urinary excretion showed increased total glycosaminoglycans (105 mg/mmol creatinine; normal for age 9-20 mg/mmol) with marked increases of dermatan and heparan sulphates. A number of lysosomal enzyme activities were measured on leucocytes, serum and cultured fibroblasts. Normal or high activities were found for alpha-iduronidase, N-acetylgalactosamine-6-sulphatase, beta-galactosidase, arylsulphatase B and beta-glucuronidase. However a marked deficiency of iduronate sulphate sulphatase activity was observed, consistent with a diagnosis of Hunter's disease. Activities were reduced to less than 2% of mean control values in the patient's leucocytes, serum and cultured fibroblasts. Normal activities were measured in samples from the father and younger sister but a partial deficiency (43% of control serum) was found in the mother. Chromosome studies on the patient revealed a partial deletion of the long arm of one X-chromosome, most probably of band Xq25, which was not inherited from either parent. Studies using BrdU indicated that the deleted X chromosome was consistently late replicating, and as a result the Hunter gene was fully expressed on the other X chromosome.
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PMID:Full expression of Hunter's disease in a female with an X-chromosome deletion leading to non-random inactivation. 310 Jan 13

Electron inactivation analysis with 16 MeV electrons was used to determine the functional target size of a number of commonly studied lysosomal hydrolases. Observed values ranged from a low of 62 000 +/- 4000 Da for beta-galactosidase to a high of 200 000 +/- 17 500 Da (mouse beta-glucuronidase). One group of lysosomal hydrolases (N-acetyl-beta-glucosaminidase, N-acetyl-beta-galactosaminidase, alpha-galactosidase, beta-mannosidase, beta-glucosidase, arylsulphatase A and sphingomyelinase) had target sizes in the range 100 000-120 000 Da, whereas alpha-glucosidase and alpha-fucosidase exist as complex multimers in the 150 000-160 000 Da range. Analysis of freeze-dried cell material showed little evidence of species (mouse versus human) variation in the functional size of most lysosomal hydrolases with the exception of beta-glucuronidase. Our findings suggest the potential usefulness of lysosomal hydrolases as endogenous marker enzymes in studies where the target size of proteins of unknown molecular mass is to be determined.
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PMID:Functional lysosomal hydrolase size as determined by radiation inactivation analysis. 315 87

Histochemical techniques were applied to salivary glands removed from adult multimmate rodents (Praomys) of either sex to detect and localize the following enzymatic activities: acid and alkaline phosphatase, arylsulphatase, ali-esterases, beta-glucuronidase, N-acetyl-betaglucosaminidase, and L-leucyl-aminopeptidase. No reaction was observed for alkaline phosphatase and glucuronidase. The glands reacted differently to the other enzymatic activities. Alkaline phosphatase and glucosaminidase were present only in one glandular type whereas arysulphatase and esterases were present in all types although demonstrating a variable staining intensity in different glands. Sharp differences in some enzymatic activities of the submandibular and parotid glands were related to the sex of the animal.
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PMID:Cytochemical demonstration of enzymatic activities of the parotid, submandibular, and sublingual glands of Praomys (Mastomys) Natalensis. 341 28

The water-soluble proteins of the rat preputial gland secretion were characterized in native and SDS-treated form on polyacrylamide gel electrophoresis. Nine major proteins were present in the secretion. One protein was a glycoprotein of molecular weight greater than 200,000 with beta-glucuronidase activity, and the other eight proteins had a molecular weight of 17,000, but with different charges. Acid phosphatase and arylsulphatase activities were present in the secretion in minor amounts. The isoelectric points of the secretory proteins ranged from 8.5 to 5.3; none of the proteins were lipoproteins, and there were no sex differences. The male and female rat urinary proteins were also characterized electrophoretically. The male rats had two different protein patterns, probably genetically determined. The female rats showed basically one urinary protein pattern, but their urines were frequently mixed with the preputial gland secretory proteins, which most likely played a part in the chemical communication. The mixing could not be correlated to daytime or estrous cycle.
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PMID:Characterization of the secretory proteins of rat preputial gland in relation to urinary proteins. 372 92

Benzarone (the debrominated metabolite of the uricosuric drug benzbromarone) has been proposed for treatment of vascular disorders. An assay was developed for the quantitation of total benzarone (conjugated and unconjugated) in plasma and urine, following oral intake of benzarone. Enzymatic hydrolysis of the samples with beta-glucuronidase/arylsulphatase, extraction, gradient elution high-performance liquid chromatography with reversed-phase columns and UV detection were used for the assay. The concentration ranges, precision and sensitivities were: 0.01-2 micrograms/ml, 3-5% and 0.01 microgram/ml, respectively, for both plasma and urine. These results were validated by gas chromatography-mass spectrometry after methylated derivatives were prepared. Enzymatic hydrolysis of plasma with pure beta-glucuronidase or arylsulphatase showed that the relative amounts of unconjugated, glucuronidated, and sulphated benzarone were 6, 12 and 82% respectively, for both plasma and urine.
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PMID:Determination of benzarone in human plasma and urine by high-performance liquid chromatography and gas chromatography-mass spectrometry. Identification of the conjugates. 378 83

Eosinophils from the blood of normal individuals were purified by centrifugation over discontinuous Percoll gradients. Eosinophil suspensions were obtained with a mean purity of 96% and a mean recovery of 64% (n = 19). When incubated with phorbol-myristate acetate, eosinophils consumed twice as much oxygen as did neutrophils from the same donors. With serum-treated zymosan, 70% and 100% of the maximal oxidative response (i.e. the response to phorbol-myristate acetate) was obtained with eosinophils and neutrophils, respectively. The calcium ionophore A23187 is a weak stimulus that triggered only 2.5% of the eosinophil and 10% of the neutrophil oxidative capacity. The response of both cell types to formyl-methionyl-leucyl-phenylalanine (fMLP) was rapid, with a maximum after 3 min. The magnitude of this eosinophil reaction was half that of neutrophils. Although the activities of the granule enzymes beta-glucuronidase and arylsulphatase were 2.5 and 6 times higher in eosinophils than in neutrophils, respectively, the exocytosis of these enzymes in response to various stimuli was lower in eosinophils. The high yield of eosinophils from our separation method enabled us to prepare eosinoplasts by centrifugation of eosinophils over discontinuous Ficoll gradients that contained cytochalasin B. Eosinoplasts are plasma membrane vesicles derived from eosinophils, filled with cytoplasm but devoid of granules and nucleus. The eosinoplasts contained 30% of the cytoplasm and plasma membrane present in intact eosinophils. Eosinoplasts still possessed a functionally intact oxidase enzyme that could be stimulated with various stimuli. Therefore, eosinoplasts may provide a valuable tool to study separately the role of the oxidase products and that of the granule contents in eosinophil functions.
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PMID:Purification of eosinophils from normal human blood, preparation of eosinoplasts and characterization of their functional response to various stimuli. 381 66


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