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Correction of the permeability defect in hereditary stomatocytosis by dimethyl adipimidate.

Abstract
The effect of imidoesters on the extraordinarily increased cation permeability of hereditary stomatocytes was evaluated in erythrocytes from a splenectomized boy with persistent hemolytic anemia. Reaction of stomatocytes with dimethyl adipimidate reduced ouabain-associated potassium loss from 15 to 1.7 and sodium gain from 22 to 2.5 meq per liter of red cells per hours. Red-cell volume, cation concentration, and deformability, previously abnormal, rapidly became normal after stomatocytes were reacted with dimethyl adipimidate. Instead of stomatocytes, normal red cells and target cells were noted. The survival (half-time) of stomatocytes treated with 51Cr-labeled dimethyl adipimidate infused into rats rendered tolerant to human erythorocytes by pretreatment with ethyl palmitate and cobra-venom factor was double that of untreated stomatocytes. Chemical modification of the defect in vitro allowed stomatocytes to regain many properites of normal erythrocytes and favorably influenced the subsequent survival of these cells in vivo.
AuthorsW C Mentzer, B H Lubin, S Emmons
JournalThe New England journal of medicine (N Engl J Med) Vol. 294 Issue 22 Pg. 1200-4 (May 27 1976) ISSN: 0028-4793 [Print] United States
PMID1264135 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, Non-P.H.S.)
Chemical References
  • Adipates
  • Imides
  • Lactates
  • Ouabain
  • Adenosine Triphosphate
  • Glucose
Topics
  • Adenosine Triphosphate (metabolism)
  • Adipates (pharmacology)
  • Adult
  • Anemia, Hemolytic, Congenital (blood, pathology)
  • Animals
  • Cell Membrane Permeability (drug effects)
  • Cell Survival (drug effects)
  • Child
  • Erythrocytes (drug effects, metabolism)
  • Erythrocytes, Abnormal (drug effects, ultrastructure)
  • Glucose (metabolism)
  • Humans
  • Imides (pharmacology)
  • In Vitro Techniques
  • Lactates (metabolism)
  • Male
  • Osmotic Fragility (drug effects)
  • Ouabain (pharmacology)
  • Rats

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