caa a22 4500 469044993 CHVBK 20180323132821.0 cr unu---uuuuu 170328e19920901xx s 000 0 eng 10.1007/BF01800017 doi (NATIONALLICENCE)springer-10.1007/BF01800017 Characterization of phosphofructokinase-deficient canine erythrocytes [Elektronische Daten] [J. Harvey, M. Pate, Y. Mhaskar, G. Dunaway] Summary: Dogs homozygously affected with muscle-type phosphofructokinase (PFK) deficiency had about 20% of normal erythrocyte PFK activity and exhibited a compensated haemolytic anaemia. Erythrocyte glucose-6-phosphate and fructose-6-phosphate concentrations were increased and dihydroxyacetone phosphate and 2,3-bisphosphoglycerate values were below normal in affected dogs. Other intermediates distal to the PFK step were not significantly below normal and fructose-1,6-bisphosphate was even above normal. Erythrocyte ATP was higher than normal in affected dogs owing to the reticulocytes present. Abnormal adenylate metabolism was demonstrated by low ATP/AMP and ADP/AMP ratios and the inability to maintain ATP content when affected erythrocytes were incubated with cyanide. Glucose-1,6-bisphosphate content was normal, and fructose-2,6-bisphosphate content in affected canine erythrocytes was higher than normal. Studies of erythrocyte PFK isozymes revealed altered enzyme kinetic properties in affected dogs which appeared to be due to the loss of the M-type subunit. SSIEM and Kluwer Academic Publishers, 1992 Harvey J. Department of Physiological Sciences, College of Veterinary Medicine, University of Florida, 32610-0144, Gainesville, FL aut Pate M. Department of Physiological Sciences, College of Veterinary Medicine, University of Florida, 32610-0144, Gainesville, FL aut Mhaskar Y. Department of Pharmacology, Southern Illinois University School of Medicine, 62794-9230, Springfield, IL, USA aut Dunaway G. Department of Pharmacology, Southern Illinois University School of Medicine, 62794-9230, Springfield, IL, USA aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 747-759 0141-8955 15:5<747 1992 15 10545 https://doi.org/10.1007/BF01800017 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800017 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Harvey J. Department of Physiological Sciences, College of Veterinary Medicine, University of Florida, 32610-0144, Gainesville, FL aut NATIONALLICENCE 700 1- Pate M. Department of Physiological Sciences, College of Veterinary Medicine, University of Florida, 32610-0144, Gainesville, FL aut NATIONALLICENCE 700 1- Mhaskar Y. Department of Pharmacology, Southern Illinois University School of Medicine, 62794-9230, Springfield, IL, USA aut NATIONALLICENCE 700 1- Dunaway G. Department of Pharmacology, Southern Illinois University School of Medicine, 62794-9230, Springfield, IL, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 747-759 0141-8955 15:5<747 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer