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:3.2.1.23 (beta-galactosidase)
14,648 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Young (60--80 days) mice of the low beta-glucuronidase strain, C3H/HeJ, showed no differences in hepatic levels of glycosaminoglycans (GAGs) when compared to the randombred, "normal" Swiss-Webster mice of the same age. However, by 12 months of age hepatic GAG is nearly twice as high in C3H/HeJ mice as in Swiss-Webster mice. Studies of beta-glucuronidase, beta-galactosidase, and N-acetyl-beta-glucosaminidase in four tissues of the two types of mice at the two ages revealed that glucuronidase was the only enzyme with lower activity in the C3H/HeJ strain.
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PMID:Glycosaminoglycan accumulation with partial deficiency of beta-glucuronidase in the C3H strain of mice. 10 77

Four hydrolases, beta-galactosidase, beta-glucuronidase, beta-N-acetylglucosaminidase and acid phosphatase were examined in red blood cells (RBC) of normal donors and patients with homozygous beta-thalassaemia. Highly sensitive fluorimetric substrates were used to determine the specific activities of these enzymes. In order to avoid contamination by lysosomal activities derived from white blood cells (WBC), the mature RBV were separated from other blood elements by cellulose chromatography. The hydrolase activities in normal RBC were detected only in their plasma membranes and were found to be considerably lower than in WBC or platelets. In thalassaemic RBC, hydrolase activities were present in both plasma membranes and in the soluble fraction. The normoblast fraction contributed most of the hydrolase activity found in these preparations, suggesting the presence of lysosomal particles in thalassaemic RBC. No differences in the enzymatic activities were found when purified membranes of mature RBC from thalassemic and normal preparations were compared. The origin and roles of these hydrolytic enzymes in normal and thalassaemic RBC membranes are not known.
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PMID:"Lysosomal" enzyme activities in red blood cells of normal individuals and patients with homozygous beta-thalassaemia. 11 37

Three lysosomal glycosidases, beta-glucuronidase (EC 3.2.1.31), beta-galactosidase (EC 3.2.1.23), and N-acetyl-beta-glucosaminidase (EC 3.2.1.30) have been investigated in bile that was freshly collected from rats through a complete bile fistula. Assay conditions have been established on the basis of appropriate kinetic studies. The biliary excretion patterns for these enzymes were found to vary considerably from rat to rat during the 24-h collection period. In a given animal, however, the three hydrolases were excreted in parallel and showed a gradual increase in activity with time, most marked after 10- 12 h of collection. 24-h biliary outputs of the three hydrolases averaged congruent with3% of their respective contents in total liver, and bile diversion had no effect on hepatic glycosidase activity or total protein content. Other enzymes known to be associated primarily with mitochondria, endoplasmic reticulum, and cell sap were also detected in bile, generally in smaller amounts. The biliary excretion of the plasma membrane markers, alkaline phosphodiesterase I and 5'-nucleotidase, however, was comparable to that of the lysosomal hydrolases. Biliary excretion of total protein was relatively constant and corresponded to 3.0% of the total hepatic protein content per day, whereas biliary bile acid secretion decreased during the first 12 h and then remained constant. Exocytic bulk discharge of hepatocyte lysosomes is proposed as the most likely mechanism for the biliary excretion of lysosomal enzymes. These results call attention to the possible pathophysiologic significance of biliary excretion of hepatic lysosomal contents as a means of residue disposal.
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PMID:Coordinate secretion of acid hydrolases in rat bile. 11 27

We have purified beta-galactosidase and beta-glucuronidase from macrophages of thioglycollate-treated mice using concanavalin A chromatography and immunoprecipitation. The apparent molecular weight of the beta-galactosidase subunit, as determined by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, changed during a long term pulse-chase experiment. Following a 1-h pulse with [3H]leucine, radiolabel was present exclusively in an Mr = 82,000 form. However, after a 3-h chase in medium containing unlabeled leucine, most label migrated at Mr = 63,000, and at 24 h, all label was in the Mr = 63,000 form. Electrophoresis of peptides produced by cyanogen bromide cleavage of immunoprecipitates demonstrated structural similarities between precursor and mature forms. A mutation in the mouse, which is known to depress the rate of synthesis of beta-galactosidase in many cell types, proportionately decreased incorporation of [3H]leucine into both the Mr = 82,000 and 63,000 forms. Therefore, by kinetic, structural, and genetic evidence, the large molecular weight beta-galactosidase is a precursor of mature macrophage enzyme. No precursor of the Mr = 75,000 subunit of beta-glucuronidase was detected.
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PMID:Biosynthesis of two lysosomal enzymes in macrophages. Evidence for a precursor of beta-galactosidase. 11 27

Melanosomes and lysosomes share several structural and biosynthetic properties. Therefore, a large number of mouse pigment mutants were tested to determine whether genes affecting melanosome structure of function might also affect the lysosome. Among 31 mouse pigment mutants, six had 1.5- to 2.5-fold increased concentrations of kidney beta-glucuronidase. Three mutants, pale ear, pearl and pallid, had a generalized effect on lysosomal enzymes since there were coordinate increases in kidney beta-galactosidase and alpha-mannosidase. The effects of these three mutations are lysosome specific since rates of kidney protein synthesis and activities of three nonlysosomal kidney enzymes were normal. Also, the mutants are relatively tissue specific in that all had normal liver lysosomal enzyme concentrations.--A common dysfunction in all three mutants was a lowered rate of lysosomal enzyme secretion from kidney into urine. While normal C57BL/6J mice daily secreted 27 to 30% of total kidney beta-glucuronidase and beta-galactosidase, secretion of these two enzymes was coordinately depressed to 1 to 2%, 8 to 9% and 4 to 5% of total kidney enzyme in the pale-ear, pearl and pallid mutants, respectively. Although depressed lysosomal enzyme secretion is the major pigment mutant alteration, the higher lysomal enzyme concentrations in pearl and pallid may be partly due to an increase in lysosomal enzyme synthesis. In these mutants kidney glucuronidase synthetic rate was increased 1.4- to 1.5-fold.--These results suggest that there are several critical genes in mammals that control the biogenesis, processing and/or function of related classes of subcellular organelles. The mechanism of action of these genes is amenable to further analysis since they have been incorporated into congenic inbred strains of mice.
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PMID:Lysosomal dysfunctions associated with mutations at mouse pigment genes. 11 47

Assay of alpha-L-iduronidase, heparin sulphamidase, N-acetyl-alpha-D-glucosaminidase, arylsulphatase B, alpha-L-fucosidase, beta-glucuronidase, beta-galactosidase and alpha-D-mannosidase in cultured cells is described. Activities in deficient fibroblast strains are compared to control fibroblast strains. The first case of Sanfilippo B in the United Kingdom is reported. A comparison of enzyme activities in cultured fibroblasts and amniotic fluid cells is made.
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PMID:Diagnosis of the mucopolysaccharidoses using cultured skin fibroblasts and amniotic fluid cells. 11 32

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

It has been known for a long time that hearing deficits may coexist in patients with thyroid disease, but without definite morphologic evidence present to correlate gland dysfunction with hearing disturbances. To clarify this relationship between thyroid dysfunction and hearing disturbances, the guinea pig was employed as an experimental model. 70 animals were thyroidectomized, and maintained in a hypothyroid state for varying periods of time. The animals were then sacrificed, and various histochemical studies then performed. These studies included analysis for glycosidase (beta-galactosidase, beta-glucuronidase, n-acetyl-beta-glucosamide), non-specific esterases, sulfatases, sulghydryl groups as well as mucous substances within the cochlea and saccus endolymphaticus of the experimental animals. Results indicated that hyaluronidase-sensitive mucous substances were increased in the scala of the inner ear. As a consequence of increased deposition of acid mucopolysaccharides, the relationship of potassium to sodium in endolymph and perilymph was found markedly altered. Marked swelling of the chambers of the inner ear was noted, and believed to represent hydropic induction by acid mucopolysaccharide-with consequent alteration of electrolyte relationships ("Electrochemical Theory").
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PMID:[Animal experiment histological-histochemical studies on the development of hearing disorders related to hypothyroidism]. 12 84

Deposition of PAS2-positive materials and thickening of the basement membrane in vascular lesions are characteristic findings in diabetes mellitus, suggesting altered metabolism of glycoprotein. Changes in the activities of the glycosidases, beta-N-acetylglucosaminidase [EC 3.2.1.30], beta-glucuronidase [EC 3.2.1.31], beta-galactosidase [EC 3.2.1.23], and beta-glucosidase [EC 3.2.1.21] were measured in various organs and the serum of diabetic rats. The activities of the first three enzymes listed above were found to be much reduced in the kidney but increased in the serum. The decreased activities of beta-glycosidases in the kidney may be one of the factors responsible for the pathogenesis of microangiopathy.
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PMID:Beta-glycosidases and diabetic microangiopathy. I. Decreases of beta-glycosidase activities in diabetic rat kidney. 13 96

The enzymic activity of acid alkaline phosphatases, of alanyl- and leucine-aminopeptidases, of beta-galactosidase and beta-glucuronidase, and of Tween-60-esterase was tested in the rabbit lung tissue during the following experimental processes: a) the sensitization with Freund adjuvant containing PPD and challenge of the delayed hypersensitivity reaction to PPD), b) the sensitization with gamma-globulin in Freund adjuvant, c) the pulmonary Arthus reaction, d) the lung granulomas induced by intravenous administration of Freund adjuvant containing heterologous lung proteins, e) the tuberculosis and silicotuberculosis under the influence of tuberculostatics. A metabolic intensification expressed by a marked increase of the tested hydrolytic enzymes was observed comparatively with controls in all these immune, granulomatous and inflammatory processes experimentally induced in the lung. The morphologic substrate of this behaviour was represented by the numerous cell proliferation and differentiations of the reticulomacrophagic type occurring during these experimental processes under the stimulation of antigenic elicitors. The latter also induced an increase of the lysosome-formation as submicroscopic site of these hydrolytic enzymes.
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PMID:Histoenzymology of the lung. II. The behaviour of lung hydrolytic enzymes during some experimental pulmonary processes. 14 Mar 19


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