Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.5.1.1 (asparaginase)
 2,695 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Homogenization of guinea pig liver in isotonic sucrose solution followed by the separation of the subcellular fractions by differential centrifugation releases the liver L-asparaginase (L-asparagine amidohydrolase, EC 3.5.1.1) activity into the supernatant fraction. Electron micrographs of the liver L-asparaginase-antibody complexes, precipitated from the clear supernatant phase by addition of L-asparaginase-specific antiserum, show membrane-liek structures and some amorphous material. The attachment of L-asparaginase to the membrane-like structures is indicated by the ferritin-labeled antibody technique. The immunoprecipitates possess low activities of 5'-nucleotidase, alkaline phosphodiesterase I, NADPH cytochrome c reductase, glucose-6-phosphatase, and acid phosphatase. This observation suggests that L-asparaginase found in the liver supernatant fraction is associated with cytomembrane components. Analysis of guinae pig serum L-asparaginase-antibody complexes is polyacrylamide gel electrophoresis in the presence of sodium dodecyl sulfate gives three distinct protein bands. These bands correspond to heavy and light chains of rabbit immunoglobulins and the L-asparaginase subunits. Analysis of the liver L-asparaginase-antibody complexes by the above procedure shows similar but more diffuse protein bands.
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PMID:Evidence for the association of L-asparaginase with cytomembrane components in the guinea pig liver soluble fraction. 81 93

One of the main criteria in the differentiation between acute lymphoblastic (ALL) and acute myeloblastic leukemias (AML) is the presence of granules in the blasts of the latter. Recently, several groups have described a form of ALL with prominent intracytoplasmatic granules (G-ALL) in the blasts. The granules in the G-ALL blasts do not contain myeloperoxidase, but sometimes have lipids that stain with Sudan black B (SBB). We describe a case of G-ALL in a five-year-old girl whose peripheral blood and bone marrow was compound of 98% lymphoblasts, 30% of which, had prominent azurophilic intracytoplasmatic granules. The granules did not have peroxidase, acid phosphatase, varies; is directly proportional to naphthyl acetate esterase. However 5% of the blasts had sudanophilic granules and 60% were positive for the periodic acid-Schiff reaction. The blasts expressed the CD10 (CALLA) and Dr antigens, and were negative for surface immunoglobulins or the CD4, CD8, or CD14, antigens. Only 18% of cells formed rosettes with sheep erythrocytes. The patient responded to vincristine, prednisone and L-asparaginase. Based on the finding we diagnosed this as a CALLA positive G-ALL. By conventional criteria this case would have been wrongly classified as AML.
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PMID:[Granular CALLA-positive acute lymphoblastic leukemia]. 210 43

A 20-year-old man was admitted to our hospital because of fever and knee joint pain on March 20, 1986. Physical examination revealed generalized lymphadenopathy and hepatomegaly. White blood cell count was 32,800 microliters with 74.4% blast cells. Bone marrow was hypercellular with 93.6% blast cells. Blast cells were weakly positive for acid phosphatase and PAS stainings but were negative for peroxidase, sudan black B and esterase stainings. Cell surface marker analysis of blast cells disclosed that they were positive for anti-HLA-DR, CD19, CD24, CD33 and CD38, but were negative for CD10 and CD20. Cytoplasmic immunoglobulin of blast cells was negative and TdT activity by immunofluorescent method was positive. Chromosomal analysis of bone marrow samples revealed normal karyotype. Therefore, this case was diagnosed as having acute lymphoblastic leukemia (L2) and achieved complete remission with LVP therapy consisting of 1-asparaginase, vincristine and prednisolone. Gene analysis of blast cells disclosed germ-line configuration of both the immunoglobulin heavy chain gene and T cell receptor beta chain gene. We speculated that the phenotype of leukemic cells might precede the genotype in some cases of acute leukemia.
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PMID:[Germ-line configuration of the immunoglobulin heavy chain gene in a case of B cell precursor acute lymphoblastic leukemia]. 255 12

The Dutch Childhood Leukemia Study Group (DCLSG) performed a phase III study-Study (ALL) V-to evaluate the effectiveness of rubidomycin in induction therapy with vincristine, prednisone, and L-asparaginase for children (0-15 years) with standard risk acute lymphoblastic leukemia (ALL) (white blood cell [WBC] counts less than 50.10(9)/L, absence of mediastinal mass, and/or cerebromeningeal leukemia). Furthermore, the influence of initial patient and disease characteristics on the outcome was analyzed. Between May 1979 and December 1982, 240 patients entered the study and were randomized into two groups: group A (n = 122) received induction treatment with vincristine (VCR), prednisone (Pred), and L-asparaginase (L-Asp); for group B (n = 118), induction therapy consisted of VCR, Pred, L-Asp, and rubidomycin (Rub). All patients subsequently underwent cranial irradiation (doses adjusted to age) in combination with intrathecal methotrexate; maintenance therapy of 6-mercaptopurine and methotrexate for 5 weeks followed by vincristine and prednisone for 2 weeks was given for 24 months. The complete remission (CR) rate was similar in both groups (94.5%). Event-free survival (EFS) 5 years after diagnosis was higher in group B (62.5 +/- 4.5%) than in group A (54.7 +/- 4.5%), although the difference is not significant (p = 0.20). A high initial WBC (greater than or equal to 10.10(9)/L), age (greater than or equal to 10 years), a low platelet count (less than 100.10(9)/L), and a positive acid phosphatase reaction of the leukemic cells were unfavorable prognostic factors (p less than 0.05). Sex, French-American-British (FAB) classification group, immunophenotype, and treatment in specialized centers did not have a significant impact on event-free survival.
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PMID:Effectiveness of rubidomycin in induction therapy with vincristine, prednisone, and L-asparaginase for standard risk childhood acute lymphocytic leukemia: results of a Dutch phase III study (ALL V). A report on behalf of the Dutch Childhood Leukemia Study Group (DCLSG). 266 46

The Dutch Childhood Leukemia Study Group performed a phase III study (Study ALL V) to evaluate the effectiveness of addition of rubidomycin to induction treatment with vincristine, prednisone and L-asparaginase in children (0-15 years) with standard risk acute lymphoblastic leukemia: WBC less than 50.10(9)/l, absence of mediastinal mass and/or cerebromeningeal leukemia. Furthermore, the influence of some initial patient- en disease-characteristics on the outcome was analysed. Between May 1979 and December 1982 240 patients entered into the study and were randomized into 2 groups: group A (n = 122) received induction treatment with vincristine, prednisone and L-asparaginase; group B (n = 118) received induction treatment with vincristine, prednisone, L-asparaginase and rubidomycin. All patients received cranial irradiation (doses adjusted to age) and intrathecal methotrexate, followed by maintenance treatment with 6-mercaptopurine and methotrexate for 5 weeks, alternated with vincristine and prednisone for 2 weeks, up to 24 months. Complete remission rate was 94% in both groups. Event-free survival at 5 years after diagnosis was higher in group B (62% +/- 4.6%) than in group A (54.2% +/- 4.6%) but the difference was not significant. A higher initial WBC, age greater than or equal to 10 years and a positive acid phosphatase reaction of the leukemic cells were unfavorable prognostic factors (p less than 0.01). Sex, FAB-morphology, immunophenotype and place of treatment (center or general hospital) were not significant factors.
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PMID:[Results of treatment of children with acute lymphatic leukemia (ALL) according to the ALL V protocol of the Netherlands Working Group on Leukemia in Children]. 328 85

Benzoyl- and isopentenoyl phosphoric triamides (BPA and IPA) strongly inhibited urease activities from jack bean, soybean, watermelon seed, Proteus mirabilis, P. rettgeri, P. vulgaris, Mycobacterium smegmatis, and Ureaplasma urealyticum. Their I50 values (the final concentration causing 50% inhibition), independent of enzyme source, were 2-21 nM, which are about 1,000-fold lower than that of caprylohydroxamic acid, one of the most potent urease inhibitors. ATP-urea amidolyase activity was inhibited 50% by BPA at a higher concentration of 0.28 mM, but was not affected by IPA even at 1.3 mM. Thirteen kinds of hydrolases (trypsin, chymotrypsin, thermolysin, leucine aminopeptidase, papain, lipase, alpha-amylase, glucuronidase, asparaginase, arylsulfatase, alkaline phosphatase, acid phosphatase, and true cholinesterase), two oxidoreductases (catalase and alcohol dehydrogenase), three transferases (glutamic-oxaloacetic aminotransferase, gamma-glutamyl transpeptidase, and arylsulfotransferase) and two kinases (pyruvate kinase and creatine kinase) were not affected at all even at 1 mM BPA and IPA. Exceptionally, pseudo-cholinesterase from human serum was inhibited by BPA and IPA, whose I50 values were 70 nM and 10 muM, respectively, using acetylthiocholine as a substrate. These values increased to 0.55 muM and 54 muM, respectively, when acetylcholine was used as a substrate. These results show that N-acylphosphoric triamides potently and specifically inhibit urease activity at concentrations of nM order.
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PMID:Specific inhibition of urease by N-acylphosphoric triamides. 384 42

Activities of several phosphohydrolases are significantly enhanced when cells of the inositol-requiring yeast, Saccharomyces uvarum ATCC 9080, are deprived of inositol. This effect is most pronounced for the external acid phosphatase and cannot be explained simply by limitation of cellular growth, because starvation for vitamins or sulphate has no effect on acid phosphatase activities. Excessive secretion of acid phosphatase by spheroplasts prepared from inositol-deficient cells is greatly reduced when the spheroplast medium is supplemented with inositol and is immediately suppressed by the addition of cycloheximide. These results together with data obtained from experiments with whole cells, employing cycloheximide and actinomycin D, point to a regulatory effect of inositol limitation at the level of transcription. The external enzymes beta-D-fructofuranosidase, alpha-D-galactosidase and L-asparaginase, and the vacuolar enzyme carboxypeptidase Y are not affected by inositol deficiency indicating that inositol deficiency has no general effect on protein secretion.
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PMID:The effect of myo-inositol deficiency on phosphatases of yeast. 608 32

Immunochemical techniques with enzymes as the antigen have grown in frequency during the last few years. These techniques have allowed evaluation of enzymes in the presence of endogenous inhibitors. Among those enzymes measured by immunochemical techniques and which have found diagnostic application, mention will be made of alkaline phosphatase (with particular reference to the intestinal, placental, and Regan isoenzymes), lactate dehydrogenase (in which renewed interest has developed due to techniques for specifically measuring the LD-1 isoenzyme), aspartate aminotransferase (of which the cytosolic and mitochondrial forms can now be independently measured by immunochemical techniques), acid phosphatase (for which a specific immunochemical assay for the prostatic enzyme has been widely introduced in diagnostic laboratories), and creatine kinase (for which a variety of immunochemical techniques to measure the M- and B-subunits are now part of standard laboratory assays). Other enzymes which will be discussed in this review include phosphohexose isomerase, amylase, ribonuclease, and lysozyme (muramidase). Finally, the use of enzymes, particularly asparaginase, in the chemotherapy of cancer will be outlined.
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PMID:Immunoassay of enzymes--an overview. 634 26

Two girls, each less than 2 yr of age, developed acute megakaryoblastic leukemia (malignant myelosclerosis). Both presented with anemia, severe thrombocytopenia, and a low percentage of blasts in their peripheral blood. Their marrow showed marked reticulin fibrosis with an increase in blasts and immature megakaryocytes. The blasts stained negatively for myeloperoxidase and Sudan Black B, but showed acid phosphatase (ACP) and alpha-naphthyl acetate esterase (ANAE) activity inhibitable by sodium fluoride. They were identified as megakaryoblasts by the platelet peroxidase reaction. Cytogenetic studies showed multiple chromosomal abnormalities in both cases. Chemotherapy with vincristine, prednisone, and L-asparaginase was without effect, while daunorubicin and cytosine arabinoside induced a complete remission in one case. The second case responded to a combination of cytosine arabinoside, daunorubicin, and 6-thioguanine. This article documents that acute megakaryoblastic leukemia occurs in early childhood and describes its clinical, pathologic, and cytogenetic features. Previous reports of childhood "myelofibrosis" are reviewed, and their possible relationship with acute megakaryoblastic leukemia is discussed.
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PMID:Acute megakaryoblastic leukemia in early childhood. 686 Jul 97

The influence of cytostatic drugs (L-asparaginase, vincristine, 6-mercaptopurine, amethopterine, prednisone) on the activity of alkaline and acid phosphatase, alpha-naphtol-acetate esterase, the content of glycogen and lipids in leukocytes of peripheral blood in patients with acute leukemia was investigated. Under the influence of anti-leukemic drugs some cytochemical reactions typically changed in different forms of acute leukemia showed tendency to normalization being sometimes more distinctive than leukocytosis or even than white blood picture. In patients who did not show any improvement during the treatment the disturbances of cytochemical reactions intensified or, sometimes, remaining unchanged. The repetition of examination of cytochemical reactions changing distinctively in the chemotherapy may simplify the treatment control by better estimation of its efficiency and give some prognostic hints.
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PMID:Influence of chemotherapy on some cytochemical reactions in patients with acute leukemia. 693 14


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