Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.6.1 (sulfatase)
 3,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Postnuclear supernates from homogenates of skeletal muscle from rats subjected to starvation, injections of Triton WR-1339, dextran-500, and dextran + corticosterone were fractionated by means of rate and isopycnic zonal centrifugation in sucrose-0.02 M KCl gradients. Zonal fractions were analyzed for protein, RNA, cytochrome oxidase, and up to six acid hydrolases. The results indicate the presence of two groups of lysosome-like particles. One group contributes approximately 95% of the cathepsin D and acid phosphatase activity and 75% of the acid ribonuclease, beta-glucuronidase, and arylsulfatase activity in muscle. It is characterized by a modal equilibrium density of 1.18 that is decreased by starvation, but is not shifted by dextran-500 or Triton WR-1339. The second group has a higher proportion of acid ribonuclease, beta-glucuronidase, and arylsulftase; the equilibrium density can be shifted by dextran-500 and Triton WR-1339. It is suggested that this group of lysosomes is derived from macrophages and other connective tissue cells, whereas the former group represents lysosome-like particles from muscle cells.
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PMID:Lysosomes in skeletal muscle tissue. Zonal centrifugation evidence for multiple cellular sources. 432 73

Histochemical procedures for PMN granule enzymes were carried out on smears prepared from normal rabbit bone marrow, and the smears were examined by light microscopy. For each of the enzymes tested, azo dye and heavy metal techniques were utilized when possible. The distribution and intensity of each reaction were compared to the distribution of azurophil and specific granules in developing PMN. The distribution of peroxidase and six lysosomal enzymes (acid phosphatase, arylsulfatase, beta-galactosidase, beta-glucuronidase, esterase, and 5'-nucleotidase) corresponded to that of azurophil granules. Progranulocytes contained numerous reactive granules, and later stages contained only a few. The distribution of one enzyme, alkaline phosphatase, corresponded to that of specific granules. Reaction product first appeared in myelocytes, and later stages contained numerous reactive granules. The results of tests for lipase and thiolacetic acid esterase were negative at all developmental stages. Both types of granules stained for basic protein and arginine. It is concluded that azurophil and specific granules differ in their enzyme content. Moreover, a given enzyme appears to be restricted to one of the granules. The findings further indicate that azurophil granules are primary lysosomes, since they contain numerous lysosomal, hydrolytic enzymes, but the nature of specific granules is uncertain since, except for alkaline phosphatase, their contents remain unknown.
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PMID:Differences in enzyme content of azurophil and specific granules of polymorphonuclear leukocytes. I. Histochemical staining of bone marrow smears. 487 49

Practical and scientific aspects of prenatal detection of genetic disorders is discussed. The indications for intrauterine detection of familial biochemical and particular chromosomal disorders require assessment of the risks of transabdominal amniocentesis and of the reliability of diagnosis. A high degree of experience in cultivating amniotic fluid cells and in performing diagnostic tests is required. The obstetrician performing the amniocentesis should be responsible for referring the family to a physician who will perform the abortion. A high-risk group for which amniocentesis may be especially important includes families in which 1 parent is a carrier of a chromosomal rearrangement and in which the woman is a known carrier of an X-linked recessive disorder so that sex determination is important. A moderate-risk group includes women who become pregnant after age 40 in which the risk of having a child with a chromosomal aberration is greater than 1%. A low-risk group includes women over 35 and women who have previously borne a child with trisomic Down's syndrome. The experience gained in over 300 patients suggests that transabdominal aminocentesis carries minimal risks to mother and fetus. Analyses of amniotic fluid obtained by amniocentesis have resulted in antenatal diagnoses of Pompe's disease (deficient alpha-1, 4-glucosidase), Tay-Sachs disease (deficient hexosaminidase A), mucopolysaccharidosis (quantitative and qualitative changes in mucopolysaccharides), methylmalonic aciduria (increased methylamlonate), and adrenogenital syndrome (increase 17-ketosteroids and pregnanetriol). There is a lack of consensus on the reliability of several of these diagnoses resulting from the direct analysis of amniotic fluid. Analyses of uncultured amniotic fluid cells have resulted in diagnoses of Pompe's disease (ultrastructural changes) and Tay-Sachs disease. Analyses of cultivated amniotic fluid cells have enabled diagnoses of galactosemia (deficient galactose-1-phosphate uridyl transferase), X-linked uric aciduria (by autoradiography), lysosomal acid phosphatase deficiency, metachromatic leukodystrophy (deficientaryl sulfatase A), mucopolysaccharidosis, cystic fibrosis (metachromatic granules), and Marfan's syndrome (metachromatic granules). Methylmalonic aciduria has been detected in utero by marternal methymalonate excretion. Adrenogenital syndrome has been detected by urinary estriol.
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PMID:Present status of amniocentesis in intrauterine diagnosis of genetic defects. 511 31

Toxicity tests on Culex pipiens fatigans with propoxur (o-isopropoxyphenyl methylcarbamate) and carbofuran (2,2-dimethyl-2,3-dihydrobenzofuranyl-7-methylcarbamate) indicated that both compounds are fast-acting insecticides. Transfer of treated larvae to fresh water results in their partial recovery from knockdown.Propoxur is metabolized by resistant and susceptible larvae by their homogenate-reduced nicotinamide-adenine dinucleotide phosphate (NADPH(2)) enzyme system and by the microsome-plus-soluble fraction of mouse-liver extracts to at least 10 organosoluble metabolites with the isopropoxy group intact. The major metabolites, which are primarily hydroxylation products or the result of degradation of these products, have tentatively been identified as: acetone plus o-hydroxyphenyl methylcarbamate, 2-isopropoxy-5-hydroxyphenyl methylcarbamate, 2-isopropoxyphenyl carbamate, and 2-isopropoxyphenyl N-hydroxymethylcarbamate. Upon incubation of water-soluble products from treated larvae with beta-glucosidase, beta-glucuronidase, aryl sulfatase and acid phosphatase, the conjugates are hydrolysed, liberating mainly hydroxylated carbamates.The results indicate that slower absorption as well as faster detoxification by hydroxylation mechanisms, together with conjugation with polar molecules and elimination, are major factors in resistance of mosquito larvae to substituted-aryl methylcarbamate insecticides.
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PMID:Carbamate resistance in mosquitos. The metabolism of propoxur by susceptible and resistant larvae of Culex pipiens fatigans. 531 55

CULTURED KB CELLS (DERIVED FROM A HUMAN ORAL CARCINOMA) GROWN IN MONOLAYERS WERE INJURED BY ONE OF THREE AGENTS: starvation by arginine deprivation or treatment with high doses of either ultraviolet radiation or x-radiation. The different agents produced changes in nucleolar structure and varying accumulations of triglyceride and glycogen. All three agents produced an increase in number and size of lysosomes. These were studied in acid phosphatase preparations, viewed by both light and electron microscopy, and, occasionally, in vital dye, esterase, and aryl sulfatase preparations. Ultrastructurally, alterations in lysosomes suggested that "residual bodies" developed in a variety of ways, i.e., from the endoplasmic reticulum, multivesicular bodies, or autophagic vacuoles. Following all three agents the endoplasmic reticulum assumed the form of "rough" or "smooth" whorls, and, after two of the agents, arginine deprivation or ultraviolet radiation, it acquired cytochemically demonstrable acid phosphatase activity. Near connections between the endoplasmic reticulum and lysosomes raise the possibility that in KB cells, at least when injured, the endoplasmic reticulum is involved in the formation of lysosomes and the transport of acid phosphatase to them.
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PMID:Effects of arginine deprivation, ultraviolet radiation, and x-radiation on cultured KB cells. A cytochemical and ultrastructural study. 532 75

During their differentiation in the bone marrow, eosinophilic leukocytes synthesize a number of enzymes and package them into secretory granules. The pathway by which three enzymes (peroxidase, acid phosphatase, and arylsulfatase) are segregated and packaged into specific granules of eosinophils was investigated by cytochemistry and electron microscopy. During the myelocyte stage, peroxidase is present within (a) all rough ER cisternae, including transitional elements and the perinuclear cisterna; (b) clusters of smooth vesicles at the periphery of the Golgi complex; (c) all Golgi cisternae; and (d) all immature and mature specific granules. At later stages, after granule formation has ceased, peroxidase is not seen in ER or Golgi elements and is demonstrable only in granules. The distribution of acid phosphatase and arylsulfatase was similar, except that the reaction was more variable and fully condensed (mature) granules were not reactive. These results are in accord with the general pathway for intracellular transport of secretory proteins demonstrated in the pancreas exocrine cell by Palade and coworkers. The findings also demonstrate (a) that in the eosinophil the stacked Golgi cisternae participate in the segregation of secretory proteins and (b) that the entire rough ER and all the Golgi cisternae are involved in the simultaneous segregation and packaging of several proteins.
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PMID:Segregation and packaging of granule enzymes in eosinophilic leukocytes. 545

In the previous paper we presented findings which indicated that enzyme heterogeneity exists among PMN leukocyte granules. From histochemical staining of bone marrow smears, we obtained evidence that azurophil and specific granules differ in their enzyme content. Moreover, a given enzyme appeared to be restricted to one of the two types. Clear results were obtained with alkaline phosphatase, but those with a number of other enzymes were suggestive rather than conclusive. Since the approach used previously was indirect, it was of interest to localize the enzymes directly in the granules. Toward this end, we carried out cytochemical procedures for five enzymes on normal rabbit bone marrow cells which had been fixed and incubated in suspension. The localization of reaction product in the granules was determined by electron microscopy. In accordance with the results obtained on smears, azurophil granules were found to contain peroxidase and three lysosomal enzymes: acid phosphatase, arylsulfatase, and 5'-nucleotidase; specific granules were found to contain alkaline phosphate. Specific granules also contained small amounts of phosphatasic activity at acid pH. Another finding was that enzyme activity could not be demonstrated in mature granules with metal salt methods (all except peroxidase); reaction product was seen only in immature granules. The findings confirm and extend those obtained previously, indicating that azurophil granules correspond to lysosomes whereas specific granules represent a different secretory product.
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PMID:Differences in enzyme content of azurophil and specific granules of polymorphonuclear leukocytes. II. Cytochemistry and electron microscopy of bone marrow cells. 569 83

Seven acid glycosidases activities have been measured in normal rat uterus during the oestrus cycle. It has been observed that alpha-fucosidase, alpha-mannosidase, beta-galactosidase, alpha-galactosidase, aryl sulfatase, acid phosphatase and hexosaminidase activities changed cyclically during the oestrus cycle. From onward oestrus to metaoestrus the enzyme activities are in their highest level, but then decline slightly towards the resting one, as the cycle progresses. It is possible that changes in glycosidases content bear a lysosomal relationship, since it is known the increase in the lysosome content of the uterus during the oestrus and metaoestrus. The increased enzyme content could be related to uterus glycoproteins secretion and degradation during the normal oestrus cycle.
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PMID:Glycosidases in the rat uterus during the oestrus cycle. 609 86

The effect of ascorbic acid on adrenal and cartilage lysosomal enzyme activity was evaluated in guinea pigs maintained on either 2.4 mg, or 150 mg of ascorbate per day. When compared to adrenal enzyme activities in animals on high dietary levels of ascorbate, the adrenals of animals on 2.4 mg of ascorbate per day expressed 50% increase in the activities of acid phosphatase, arylsulfatase A, and arylsulfatase B (p less than 0.02). In cartilage, the activity of acid phosphatase was significantly elevated in animals on low dietary levels of ascorbate (p less than 0.01), but arylsulfatase A and arylsulfatase B activities were not affected by varying the dietary intake of ascorbate. These data indicate an inverse relationship between tissue vitamin C concentrations and lysosomal enzyme activities.
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PMID:Effect of ascorbate on lysosomal enzyme activities in guinea pig cartilage and adrenals. 613 Oct 46

Lysosomal hydrolases produce degradation of glomerular basement membrane and may play a key role in catabolism of glycoproteins of extracellular matrix in glomeruli. Therefore we investigated activities of some lysosomal enzymes and stability of lysosomes in glomeruli of normal and nephrotic rats. Nephrosis was induced in rats by single injections of puromycin aminonucleoside. In glomeruli from nephrotic rats we found lower activities of beta-fucosidase and arylsulfatase, but activity of acid phosphatase was higher compared with control rats. Osmotic stability of lysosomes measured by release of beta-glucuronidase was decreased in nephrotic rats. Abnormal activity of lysosomal enzymes and altered physiology of lysosomes in glomeruli may be a pathogenic factor in the altered glycoprotein metabolism in nephrotic syndrome and perhaps also in other glomerular diseases.
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PMID:Glomerular lysosomal enzymes in aminonucleoside nephrosis. 617 16


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