Abstract

Our laboratory will continue to explore the metabolism and enzymology of human blood cells. We will continue to attempt to define molecular lesions in hemolytic anemias of both acquired and genetic origin. Where these are defined, the biochemistry will be correlated with the clinical and hematologic manifestations of the disorder, its mode of genetic transmission when this is applicable, and findings in family members. We will continue to develop appropriate methodology for these metabolic studies; not only red cells but human white blood cells and possibly blood platelets will be investigated if the clinical disorder renders this appropriate.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
5R01HL012944-18
Application #
3334548
Study Section
Hematology Subcommittee 2 (HEM)
Project Start
1978-01-01
Project End
1987-12-31
Budget Start
1987-01-01
Budget End
1987-12-31
Support Year
18
Fiscal Year
1987
Total Cost
Indirect Cost

  Institution

Name
University of California Los Angeles
Department
Type
Schools of Medicine
DUNS #
119132785
City
Los Angeles
State
CA
Country
United States
Zip Code
90095

  Publications

Paglia, D E (1993) Acute episodic hemolysis in the African black rhinoceros as an analogue of human glucose-6-phosphate dehydrogenase deficiency. Am J Hematol 42:36-45
Valentine, W N; Paglia, D E (1990) Erythroenzymopathies and hemolytic anemia: the many faces of inherited variant enzymes. J Lab Clin Med 115:12-20
Paglia, D E; Valentine, W N; Nakatani, M et al. (1989) AMP deaminase as a cell-age marker in transient erythroblastopenia of childhood and its role in the adenylate economy of erythrocytes. Blood 74:2161-5
Valentine, W N; Paglia, D E; Nakatani, M et al. (1989) Inhibition of adenylate kinase by P1,P5-di(adenosine 5') pentaphosphate in assays of erythrocyte enzyme activities requiring adenine nucleotides. Am J Hematol 32:143-5
Valentine, W N; Herring, W B; Paglia, D E et al. (1988) Pyruvate kinase Greensboro. A four-generation study of a high K0.5s (phosphoenolpyruvate) variant. Blood 72:1054-9
Paglia, D E; Valentine, W N; Brockway, R A et al. (1987) Substrate specificity and pH sensitivity of deoxyribonucleotidase and pyrimidine nucleotidase activities in human hemolysates. Exp Hematol 15:1041-7
Valentine, W N; Toohey, J I; Paglia, D E et al. (1987) Modification of erythrocyte enzyme activities by persulfides and methanethiol: possible regulatory role. Proc Natl Acad Sci U S A 84:1394-8
Ravindranath, Y; Paglia, D E; Warrier, I et al. (1987) Glucose phosphate isomerase deficiency as a cause of hydrops fetalis. N Engl J Med 316:258-61
Paglia, D E; Valentine, W N; Miller, R E et al. (1986) Acute intravascular hemolysis in the black rhinoceros: erythrocyte enzymes and metabolic intermediates. Am J Vet Res 47:1321-5
Paglia, D E; Valentine, W N; Nakatani, M et al. (1986) Mechanisms of adenosine 5'-monophosphate catabolism in human erythrocytes. Blood 67:988-92

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