Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.5.4.4 (adenosine deaminase)
 5,136 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two children with acute lymphoblastic leukemia (ALL), whose lymphoblasts lacked terminal deoxynucleotidyl transferase (TdT) by both enzyme and fluorescent antibody assay, responded poorly or not at all to vincristine and prednisone. Both patients had high presenting white counts and mixed L1-L2 morphology. Lymphoblasts from one patient, an adolescent boy with a mediastinal mass, possessed surface membrane receptors for sheep red cells (E) and for complement (EAC) and had elevated adenosine deaminase activity (ADA). Lymphoblasts from a 2.5-yr-old boy without a mediastinal mass did not form E or EAC rosettes and did not express the la-like antigen or carry surface immunoglobulin. The poor response to therapy and absence of TdT were associated with a lymphoblast phenotype suggestive of a highly differentiated T-cell-derived line in one instance and an undifferentiated cell in the other instance. It is postulated that absence of TdT may predict poor therapeutic efficacy of vincristine and prednisone in acute lymphoblastic leukemia in childhood. The absence of TdT may correlate with other developmental characteristics of lymphoblasts, such as altered function or low numbers of glucocorticoid receptors or resistance to lysis by steroid drugs. Determination of many parameters of lymphoblast phenotype at diagnosis to characterize the nature of the malignant cells more precisely may ultimately enhance our understanding of, and improve therapy for, the group of leukemic children who fail to respond to standard regimens.
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PMID:Absence of terminal transferase may predict failure of remission induction in childhood ALL. 657 97

The leukemic cells in chronic lymphatic leukemia (CLL) patients have been studied prior to theory with a panel of immunological markers. Cells were assayed for the presence of receptors for sheep erythrocytes (E active and total rosettes), C3d component of complement (EAC rosettes), mouse erythrocytes (M rosettes), some of them also for surface membrane immunoglobulins (SmIg). In vitro 24 h cultures without mitogen (detection of spontaneous DNA synthesis) or 72 h cultures with phytohemagglutinin (PHA) were also performed. These conventional immunological markers and functional lymphocyte characteristics have been correlated with enzyme activities of adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP). Electrophoretic patterns of radiolabeled proteins under denaturing conditions (SDS-PAGE) have also been determined in some patients of this group. Phenotypic surface characterization of blood elements of all CLL patients studied revealed their B origin, with increased values of EAC rosette forming cells and especially increased values of M rosette forming cells. Significantly decreased values of both, ADA and PNP, were found in all the cases. Electrophoretic patterns of radiolabeled surface proteins from cells of CLL patients were essentially similar within the group with characteristically strongly radiolabeled glycoproteins gp44--HLA heavy chain and glycoproteins gp29, gp35--Ia-like or HLA-DR antigen.
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PMID:Some immunological and biochemical markers in chronic lymphatic leukemia patients. 681 48