Extraction in the presence of sodium hydroxide and cysteine allows estimates of NADPH and total NADP in human red cells without the erroneously high values of NADP+ obtained with earlier methods. An application of this technique to G6PD-deficient cells reveals that most of the nucleotide is in the oxidized form. In contrast, normal red cells have nearly all of the nucleotide in the reudced form. In addition to providing information concerning the intracellular regulation of the hexose monophosphate shunt, these findings support the concept that G6PD deficiency is a product-deficiency disorder.
H N Kirkman, G D Gaetani, E H Clemons, C Mareni
Title and authors | Publication | Year |
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Stabilization of glucose-6-phosphate dehydrogenase oligomers enhances catalytic activity and stability of clinical variants.
Garcia AA, Mathews II, Horikoshi N, Matsui T, Kaur M, Wakatsuki S, Mochly-Rosen D |
The Journal of biological chemistry | 2022 |
Semisynthetic sensor proteins enable metabolic assays at the point of care
Q Yu, L Xue, J Hiblot, R Griss, S Fabritz, C Roux, PA Binz, D Haas, JG Okun, K Johnsson |
Science | 2018 |
A Multi-scale Computational Platform to Mechanistically Assess the Effect of Genetic Variation on Drug Responses in Human Erythrocyte Metabolism
N Mih, E Brunk, A Bordbar, BO Palsson, M Punta |
PLoS computational biology | 2016 |
Determination of Reduced Nicotinamide Adenine Dinucleotide Phosphate Concentration Using High-Performance Liquid Chromatography with Fluorescence Detection: Ratio of the Reduced Form as a Biomarker of Oxidative Stress
Y Ogasawara, M Funakoshi, K Ishii |
Biological & Pharmaceutical Bulletin | 2009 |
Dramatic reduction of erythrocyte glucose‐6‐phosphate dehydrogenase activity in athletes participating in the ultradistance foot race “Spartathlon”
KH Schulpis, M Tsironi, K Skenderi, C Lazaropoulou, N Parthimos, G Reclos, E Goussetis, S Tsakiris, I Papassotiriou |
Scandinavian Journal of Clinical & Laboratory Investigation | 2008 |
Advances in Enzymology - and Related Areas of Molecular Biology
H Waelsch |
Advances in Enzymology and Related Areas of Molecular Biology | 2006 |
In silico model-driven assessment of the effects of single nucleotide polymorphisms (SNPs) on human red blood cell metabolism
N Jamshidi, SJ Wiback, BØ B |
Genome research | 2002 |
Oxidative Stress and the Mobilisation of Arachidonic Acid in Stimulated Human Platelets: Role of Hydroxyl Radical
M Muller, TC Sorrell |
Prostaglandins | 1997 |
Polyol pathway activation and glutathione redox status in non—insulin-dependent diabetic patients
MC Bravi, P Pietrangeli, O Laurenti, S Basili, M Cassone-Faldetta, C Ferri, GD Mattia |
Metabolism | 1997 |
Mutants that show increased sensitivity to hydrogen peroxide reveal an important role for the pentose phosphate pathway in protection of yeast against oxidative stress
H Juhnke, B Krems, P Kötter, |
MGG Molecular & General Genetics | 1996 |
Deficiency of two red-cell flavin enzymes in a population in Sardinia: was glutathione reductase deficiency specifically selected for by malaria?
BB Anderson, L Corda, GM Perry, D Pilato, M Giuberti, C Vullo |
The American Journal of Human Genetics | 1995 |
Equine Glucose-6-phosphate Dehydrogenase Deficiency
SL Stockham, JW Harvey, DA Kinden |
Veterinary pathology | 1994 |
HPLC determination of oxidized and reduced pyridine coenzymes in human erythrocytes
V Micheli, HA Simmonds, M Bari, G Pompucci |
Clinica Chimica Acta | 1993 |
Molecular basis of chronic non-spherocytic haemolytic anaemia: a new G6PD variant (393 ArgâHis) with abnormal KmG6P and marked in vivo instability
S Filosa, V Calabrò, D Vallone, V Poggi, P Mason, D Pagnini, F Alfinito, B Rotoli, G Martini, L Luzzatto, G Battistuzzi |
British Journal of Haematology | 1992 |
Response of Pseudomonas aeruginosa to pyocyanin: mechanisms of resistance, antioxidant defenses, and demonstration of a manganese-cofactored superoxide dismutase
DJ Hassett, L Charniga, K Bean, DE Ohman, MS Cohen |
Infection and immunity | 1992 |
International Review of Cytology
RA Miller |
International Review of Cytology | 1991 |
Bound and unbound pyridine dinucleotides in normal and glucose-6-phosphate dehydrogenase-deficient erythrocytes
L Canepa, AM Ferraris, M Miglino, GF Gaetani |
Biochimica et Biophysica Acta (BBA) - General Subjects | 1991 |
The levels of adenine nucleotides and pyridine coenzymes in red blood cells from the newborn, determined simultaneously by HPLC
M Formato, B Masala, GD Luca |
Clinica Chimica Acta | 1990 |
A new glucose-6-phosphate dehydrogenase variant with congenital nonspherocytic hemolytic anemia (G6PD Genova): Biochemical characterization and mosaicism expression in the heterozygote
GF Gaetani, S Galiano, C Melani, M Miglino, GL Forni, G Napoli, L Perrone, AM Ferraris |
Human Genetics | 1990 |
Red-cell GSH regeneration and glutathione reductase activity in G6PD variants in the Ferrara area
BB Anderson, G Carandina, M Lucci, GM Perry, C Vullo |
British Journal of Haematology | 1987 |
Glutathione reductase activity and its relationship to pyridoxine phosphate activity in G6PD deficiency
BB Anderson, JE Clements, GM Perry, C Studds, C Vullo, G Salsini |
European Journal of Haematology | 1987 |
Spectrophotometric determination of oxidized and reduced pyridine nucleotides in erythrocytes using a single extraction procedure
CR Zerez, SJ Lee, KR Tanaka |
Analytical Biochemistry | 1987 |
A very fast ion-pair reversed-phase HPLC method for the separation of the most significant nucleotides and their degradation products in human red blood cells
V Stocchi, L Cucchiarini, F Canestrari, MP Piacentini, G Fornaini |
Analytical Biochemistry | 1987 |
The function of catalase-bound NADPH
HN Kirkman, S Galiano, GF Gaetani |
The Journal of biological chemistry | 1987 |
Regional Distributions of Thiobarbituric Acid-Reactive Products, Activities of Enzymes Regulating the Metabolism of Oxygen Free Radicals, and Some of the Related Enzymes in Adult and Aged Rat Brains
Y Mizuno, K Ohta |
Journal of Neurochemistry | 1986 |
Effects of nine synthetic putative metabolites of primaquine on activity of the hexose monophosphate shunt in intact human red blood cells in vitro
JK Baird, GJ McCormick, CJ Canfield |
Biochemical Pharmacology | 1986 |
Oxidative activity of hydroxylated primaquine analogs
JK Baird, DE Davidson, JE Decker-Jackson |
Biochemical Pharmacology | 1986 |
Glucose-6-phosphate Dehydrogenase
AD Flora, A Morelli, M Grasso |
Glucose-6-phosphate Dehydrogenase | 1986 |
NADP-binding proteins causing reduced availability and sigmoid release of NADP+ in human erythrocytes
HN Kirkman, GF Gaetani, EH Clemons |
The Journal of biological chemistry | 1986 |
6-Phosphogluconolactonase deficiency, a hereditary erythrocyte enzyme deficiency: possible interaction with glucose-6-phosphate dehydrogenase deficiency
E Beutler, W Kuhl, T Gelbart |
Proceedings of the National Academy of Sciences | 1985 |
Glucose 6-Phosphate Dehydrogenase Deficiency Genetic, Pathophysiologic and Therapeutic Aspects
MA El-Hazmi, AS Warsy |
Annals of Saudi medicine | 1985 |
Recent aspects on glucose-6-phosphate dehydrogenase
GF Gaetani, E Salvidio |
Carlsberg Research Communications | 1984 |
Methylene blue-mediated hexose monophosphate shunt stimulation in human red blood cells in vitro: Independence from intracellular oxidative injury
JK Baird |
International Journal of Biochemistry | 1984 |
Xylitol mediated amelioration of acetylphenylhydrazine-induced hemolysis in rabbits
WA Ukab, J Sato, YM Wang, J Eys |
Metabolism | 1981 |
A Contradiction Between in vivo and in vitro Activities of Normal and Variant Glucose 6-Phosphate Dehydrogenase
A Yoshida |
Hemoglobin | 1980 |
Favism: Erythrocyte Metabolism during Haemolysis and Reticulocytosis
GF Gaetani, C Mareni, E Salvidio, S Galiano, T Meloni, P Arese |
British Journal of Haematology | 1979 |
Abnormal erythrocyte metabolism in hepatic disease: Effects of NADP repletion
JR Smith, NE Kay, AJ Gottlieb, FA Oski |
American Journal of Hematology | 1979 |
In vitro correction of erythrocyte glucose 6-phosphate dehydrogenase (G6PD) deficiency
A Morelli, U Benatti, F Salamino, B Sparatore, M Michetti, E Melloni, S Pontremoli, AD Flora |
Archives of Biochemistry and Biophysics | 1979 |
Partial deficiency of red cell 6-phosphogluconate dehydrogenase: A family study
F Ajmar, G Lamedica, C Garr, R Ravazzolo, M Sessarego, A Campostano |
Human Genetics | 1979 |
Effect of haemolysis on the hexose monophosphate pathway in normal and in glucose-6-phosphate dehydrogenase-deficient erythrocytes
S Galiano, C Mareni, GF Gaetani |
Biochimica et Biophysica Acta (BBA) - Bioenergetics | 1978 |
Hemolytic Anemia in Disorders of Red Cell Metabolism
E Beutler |
1978 | |
Parallel occurrence of oxidant-sensitivity and decreased inhibition by NADPH in G-6-PD Lublin and G-6-PD Poznań
AL Pawlak, D Rożynkowa |
Human Genetics | 1977 |
Drug induced hemolysis
YM Wang |
General Pharmacology: The Vascular System | 1977 |
Radioimmunoassay and chemical properties of glucose-6-phosphate dehydrogenase and of a specific NADP(H)-binding protein (FX) from human erythrocytes
A Flora, A Morelli, M Frascio, G Corte, B Curti, M Galliano, C Gozzer, L Minchiotti, C Mareni, G Gaetani |
Biochimica et Biophysica Acta (BBA) - General Subjects | 1977 |
Haemolytic Effect of Two Sulphonamides Evaluated by a New Method
GD Gaetani, C Mareni, R Ravazzolo, E Salvidio |
British Journal of Haematology | 1976 |
NADP+ and NADPH in glucose-6-phosphate dehydrogenase-deficient erythrocytes under oxidative stimulation
C Mareni, GF Gaetani |
Biochimica et Biophysica Acta (BBA) - Bioenergetics | 1976 |
Formaldehyde-Induced Hemolysis during Chronic Hemodialysis
EP Orringer, WD Mattern |
New England Journal of Medicine | 1976 |
Letter: Glucose-6-phosphate deficiency and inhibition by NADPH: a self-contradictory argument
H Kirkman, G Gaetani |
Science | 1975 |
Response : Glucose-6-Phosphate Deficiency and Inhibition by NADPH: a Self-Contradictory Argument
A Yoshida |
Science | 1975 |