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)

Bovine factor VIII aggregates human platelets either in a strong single wave at high concentration (10 mug/ml platelet suspension) or in two waves at low concentration (0.2-I mug/ml). The strong single wave of aggregation is not associated with release of [14C]serotonin or beta-glucuronidase; the high concentration does not induce retraction of reptilase-clotted platelet-rich plasma. Wtih the low concentration, relase of [14C]serotonin is observed just prior to the onset of the second wave of aggregation; release of beta-glucuronidase does not occur at any moment. The low concentration of bovine factor VIII induces moderate retraction of reptilase-clotted platelet-clotted platelet-rich plasma, which is inhibited by acetylsalicylic acid, indomethacin and apyrase, indicating that it is a consequence of release of platelet adenosine-5'-diphosphate. It has previously been suggested tht carbohydrate groups are involved in the human platelet-bovine factor VIII interaction, since galactose oxidase and periodate oxidation abolish the platelet aggregating activity of bovine factor VIII. The present study shows that these oxidizing substances also induce a degradation of bovine factor VIII, so that the exact role of carbohydrate groups in the aggregation process remains to be established.
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PMID:Factor VIII and human platelet aggregation. II. Characteristics of aggregation of human platelets by bovine factor VIII. 98 97

The cytochemical features of blast cells were studied in 45 patients with blastic phase of chronic granulocytic leukaemia. Various degrees of Sudan black B positivity was characteristic of myeloblastic transformation (23 patients), while in the medullary blast cells of nine patients with myelomonocytic transformation the alpha-naphthyl-acetate esterase showed intensive activity. In two cases the demonstrability of beta-thromboglobulin and factor VIII-related antigen in blast cells showing otherwise PAS, acid phosphatase and alpha-naphthyl-acetate esterase activity referred to megakaryocytic transformation. In six patients with lymphoid blast crisis proliferation of the Sudan negative blast cells with different granular PAS, acid phosphatase and/or beta-glucuronidase positivity was demonstrated. In five cases the cytochemical findings of leukaemic cells indicated biphenotypic and mixed transformation, respectively.
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PMID:Cytochemical studies in the blastic transformation of chronic granulocytic leukaemia. 247 28

Gene therapy holds great promise for the treatment of a variety of inherited diseases, including hemophilia A and mucopolysaccharidosis type VII (MPS VII). In both these disorders, subnormal levels of replacement protein have therapeutic effects. Thus we hypothesized that transduction of a small proportion of cells by feline immunodeficiency virus (FIV)-based lentiviral vectors might provide sufficient levels of transgene expression for phenotypic correction. We intravenously injected replication-deficient FIV-based vectors encoding either human factor VIII or human beta-glucuronidase into factor VIII-deficient or beta-glucuronidase-deficient mice, respectively. This route of delivery targeted multiple organs, with the liver as the primary transduction site. In the hemophilia A mice, factor VIII expression persisted for the duration of the experiments (approximately 5 months), and recipient mice survived an otherwise lethal bleeding episode (tail-clipping). In mucopolysaccharidosis type VII mice, substantial beta-glucuronidase activity was detected in several tissues and corresponded with marked reduction of lysosomal storage in liver and spleen. These findings indicate that gene transfer with FIV-based lentiviral vectors can permanently introduce transgenes into a sufficient number of hepatocytes for long-term therapeutic effect and suggest potential clinical value of FIV-based lentiviral vectors for treatment of hemophilia A and MPS VII.
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PMID:In vivo treatment of hemophilia A and mucopolysaccharidosis type VII using nonprimate lentiviral vectors. 1140 98