Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.21 (beta-glucosidase)
 3,280 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Brain enzymes activities that are likely to be involved in the catabolism of gangliosides were determined in controls (20% casein diet), postnatally undernourished (6.5% casein diet) and undernourished rats treated with either thyroxine or hydrocortisone, at 21 days of age. Postnatal undernutrition imposed by maternal protein deficiency during lactation resulted in a decrease in body weight and brain wet weight of the pups at 21 days of age. Administration of thyroxine or hydrocortisone to the undernourished pups every day between 16 and 21 days caused a further decrease in the body weight of the pups. On the other hand, the wet weight of brain showed a slight gain following hydrocortisone treatment. Postnatal undernutrition during lactation elevated the activities of beta-glucosidase, beta-galactosidase, beta-hexosaminidase and sialidase in rat brain. Short-term administration of thyroxine or hydrocortisone to the undernourished pups, every day between 16 and 21 days postnatal age decreased the enzyme activities. However, reversal of the increased enzyme activities to the normal lower level was completed only in the case of undernourished pups treated with hydrocortisone.
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PMID:Neonatal undernutrition and short term administration of hydrocortisone and thyroxine: effects on rat brain hydrolases. 850 8

The present study examines effects of continuous exposure to alcohol during gestation, lactation and postweaning periods and rehabilitation on gangliosides and their catabolizing enzymes in whole brain (WB), cerebrum (C), cerebellum (CB) and brain stem (BS) of 63-day-old rats. Continuous exposure to alcohol was found to cause significant deficits in the body and brain weights. On the other hand, the concentration of total ganglioside in whole brain, cerebrum, cerebellum and brain stem showed an increase following exposure to alcohol. In agreement with the increased ganglioside concentration the activities of sialidase, beta-galactosidase, beta-glucosidase and beta-hexosaminidase, which are likely to be involved in the catabolism of gangliosides, showed reductions due to alcohol. Alcohol was also found to alter the proportions of individual gangliosides and the changes were found to be region-specific. However; the alcohol-induced alterations were reversed, at least to some extent, upon abstinence from alcohol. Body weights of control (CT), alcoholic (AC) and rehabilitated (AR) rats were 164 +/- 2, 107 +/- 7 and 139 +/- 3 (mean +/- S.E.M.), respectively. Decrease in tissue weight was significant in whole brain, cerebrum and brain stem but not in cerebellum. In AR rats significant deficits in tissue weights persisted in cerebrum and almost a complete recovery was observed in brain stem. On the other hand, the increase in the concentration of gangliosides in WB, C, CB and BS of AC rats amounted to 23, 19, 19 and 53% of controls, respectively. The corresponding values for the AR rats were 12, 14, 3 and 5%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Alterations and recovery of rat brain gangliosides and glycosidases following long-term exposure to alcohol and rehabilitation during development. 851 32

Lysosomal storage disease is one of the inborn errors of metabolism caused by a deficiency of lysosomal acid hydrolase activity. We describe here the details of screening methods for the diagnosis of this disorder. It is definitely important to perform both enzyme assay of acid hydrolases and the detection of accumulated materials in patient's tissues. Leukocytes (lymphocytes), serum or plasma, and cultured skin fibroblasts are commonly used as the enzyme source for the assay. Although most lysosomal storage diseases can be diagnosed using leukocytes as the enzyme source, enzymatic activities of beta-glucosidase and sialidase in leukocytes are sometimes normal even in patients. At present, the most reliable enzyme source is considered to be cultured skin fibroblasts. Nevertheless, we should remind that we cannot detect a deficiency of galactocerebroside beta-galactosidase activity even using fibroblasts, if we use synthetic substrate. Natural substrates should be employed for the correct diagnosis and for the study of the nature of patient's enzyme. Deficiency of the enzymatic activity in patients should be confirmed by the demonstration of accumulated materials due to the enzyme defect in patient's tissues and urine. The accumulation of mucopolysaccharides and oligosaccharides in urine is obvious in patients with mucopolysaccharidoses and mucolipidoses, respectively. In case of sphingolipidoses, rectal biopsy specimen and blood could be a target of the investigation. In final, the choice of these screening methods should be made solely based on the detailed clinical manifestation of patients.
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PMID:[Screening methods for the diagnosis of lysosomal storage disease]. 857 38

The developmental profiles of sialidase, beta-galactosidase, beta-hexosaminidase and beta-glucosidase were compared to those of the gangliosides in rat brain and spinal cord. The glycosidase activities (enzyme units/g wet tissue), except beta-galactosidases, were found to be higher in brain than spinal cord, in adult rats. Among the hydrolases, beta-hexosaminidase showed a higher level of activity in both brain and spinal cord. In brain, the hydrolases, except beta-glucosidase, followed a similar developmental pattern, showing an increase from birth to 21 days, and then decreased to adult values by day 90. In the spinal cord, sialidase, beta-galactosidase, pH 3.1, and beta-hexosaminidase activities increased from birth to 21 days, reaching peak values. These activities then declined to adult values by 90 days of age. However, beta-galactosidase, pH 4.5, and beta-glucosidase activities showed a peak at day 14. Brain total ganglioside concentration (microgram N-acetylneuraminic acid/g tissue) increased slowly between birth and 7 days of age, followed by a rapid phase of increase to attain a peak value by day 21. The concentration of total gangliosides in the spinal cord is less when compared to the brain. The proportions of individual gangliosides in the central nervous system also vaired during development. The rapid phase of increase in enzyme activities between 0-7 and 14-21 days and a decrease thereafter is consistent with the turnover rate of gangliosides, which in rat brain is reported to be highest between 10 and 20 days.
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PMID:Postnatal development of glycosidases and gangliosides in the rat central nervous system. 888 81

Although the neuronal ceroid-lipofuscinoses (NCLs) are often referred to as lysosomal storage disorders, information on brain lysosomal hydrolases in NCLs is not available. We have determined the specific activities of several acid hydrolases in postmortem brain gray matter of infantile (INCL), late infantile (LINCL), juvenile (JNCL), and adult (ANCL) forms of NCL, patients affected with other neurological disorders (ON), and normal controls. The specific activities of beta-hexosaminidase A and B were significantly high in JNCL gray matter, whereas in LINCL, the increase is significant only in beta-hexosaminidase compared to the controls. A significant increase in the activities of alpha-mannosidase, beta-glucuronidase, and acid phosphatase was also observed in LINCL and JNCL patients compared to the control values. beta-galactosidase activity was also found to be elevated in JNCL brains over the controls. In contrast, activities of beta-glucosidase and sialidase appeared to be lowered in INCL and LINCL. On the other hand, alpha-fucosidase, beta-mannosidase, and sulfatase were unaffected in NCLs brains. Thus, the present data indicate NCLs related abnormalities in some of the acid hydrolases in brain gray matter, which are primarily glycoproteins of lysosomal origin. These data in conjuction with the reported association of sphingolipid activator proteins (SAP) A and D and lysosomal glycoproteins with NCL storage bodies imply abberations in the glycoconjugate metabolism and lysosomal function.
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PMID:Brain lysosomal hydrolases in neuronal ceroid-lipofuscinoses. 897 94

Total glycolipid content of Saccharomyces cerevisiae cells increased in ethanol-treated yeast cells. Sialic acid and hexosamine contents of glycolipids from ethanol-treated cells decreased, whereas those of hexoses increased. Increased sialidase activity in the presence of ethanol may be responsible for the decrease in sialic acid content of glycolipids. The saccharide moieties of glycolipids of S. cerevisiae consisted of fucose, mannose, galactose, and glucose. Ethanol treatment of yeast cells caused an increase in glucose and a decrease in galactose content of glycolipids. The changes in glucose content can be related to changes in beta-glucosidase activity under alcohol stress. The content of cerebrosides, sulfatides, and monoglucosyldiglycerides was enhanced following ethanol treatment. An increase in cerebroside as well as in sulfatide content during alcohol stress might play an important role in stabilizing the membrane both physically and structurally. Such variations in glycolipid content and composition of S. cerevisiae cells may represent an adaptive response to ethanol stress.
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PMID:Ethanol-induced changes in glycolipids of Saccharomyces cerevisiae. 1663 81


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