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Research Article Free access | 10.1172/JCI106539
Division of Hematology of the Department of Medicine, Children's Hospital Medical Center and Beth Israel Hospital, Boston, Massachusetts 02115
Microcirculation Laboratory of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115
Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115
Find articles by Mentzer, W. in: JCI | PubMed | Google Scholar
Division of Hematology of the Department of Medicine, Children's Hospital Medical Center and Beth Israel Hospital, Boston, Massachusetts 02115
Microcirculation Laboratory of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115
Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115
Find articles by Baehner, R. in: JCI | PubMed | Google Scholar
Division of Hematology of the Department of Medicine, Children's Hospital Medical Center and Beth Israel Hospital, Boston, Massachusetts 02115
Microcirculation Laboratory of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115
Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115
Find articles by Schmidt-Schönbein, H. in: JCI | PubMed | Google Scholar
Division of Hematology of the Department of Medicine, Children's Hospital Medical Center and Beth Israel Hospital, Boston, Massachusetts 02115
Microcirculation Laboratory of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115
Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115
Find articles by Robinson, S. in: JCI | PubMed | Google Scholar
Division of Hematology of the Department of Medicine, Children's Hospital Medical Center and Beth Israel Hospital, Boston, Massachusetts 02115
Microcirculation Laboratory of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115
Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115
Find articles by Nathan, D. in: JCI | PubMed | Google Scholar
Published March 1, 1971 - More info
Radioisotope studies of bilirubin turnover, ferrokinetics, and red cell survival (51Cr) in a patient with erythrocyte PK deficiency have provided evidence for prompt reticulocyte sequestration and destruction by the reticuloendothelial system. More mature erythrocytes appeared to survive well despite their deficiency of PK. PK-deficient reticulocytes, dependent upon oxidative phosphorylation for ATP production, are exquisitely sensitive to cyanide- or nitrogen-induced mitochondrial inhibition. If oxidative phosphorylation is unavailable, ATP levels decline rapidly, producing alterations in the cell membrane which allow massive losses of potassium and water. The result is a shrunken, spiculated, viscous cell whose rheologic properties would favor its sequestration by the reticuloendothelial system. Those reticulocytes with particularly low levels of PK exhibit very low glycolytic rates and thus are uniquely reliant upon oxidative phosphorylation. Other reticulocytes, better endowed with PK activity, can meet the increased ATP requirements of young erythrocytes. Upon reaching maturity, such cells have diminished ATP needs and can, therefore, survive despite their enzyme deficiency.
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