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Erythrocyte Membrane Studies in Familial Hypokalemic Periodic Paralysis

Erythrocyte Membrane Studies in Familial Hypokalemic Periodic Paralysis Abstract • A study of RBC membrane functions was performed in four patients suffering from familial hypokalemic periodic paralysis who had permanent muscular weakness. Electrophoretograms of membrane proteins, cell deformability, calciumpromoted potassium efflux, calcium-ATPase activity, and endogeneous phosphorylation of membrane proteins were all within the normal range. These results are compared with similar studies performed in myotonic and Duchenne-type dystrophies, in which abnormalities in the RBC membrane have been described. The results do not support the theory of RBC involvement in hypokalemic periodic paralysis. However, this does not imply that the muscle cell membrane is not involved in the underlying pathological process in this disorder. References 1. Oppenheim H: Neue Mitteilungen über den von Professor Westphal beschriebenen Fall von periodischer Lähmung aller vier Extremitäten . Charité Ann 16:350-372, 1891. 2. Engel AG, Lambert EH: Calcium activation of electrically inexcitable muscle fibers in primary hypokalaemic periodic paralysis . Neurology 19:851-858, 1969.Crossref 3. Bernhardt M: Notiz über die familiäre Form der Dystrophia muscularis progressiva und deren Combination mit periodisch auftretender paroxysmaler Lähmung . Dtsch Z Nervenheilk 8:111-118, 1895.Crossref 4. Rowland LP: Pathogenesis of muscular dystrophies . Arch Neurol 33:315-321, 1976.Crossref 5. Biemond A, Polak-Daniels A: Familial periodic paralysis and its transition into spinal muscular atrophy . Brain 57:91-108, 1934.Crossref 6. MacDonald RD, Rewcastle NB, Humphrey JG: Myopathy of hypokalaemic periodic paralysis . Arch Neurol 20:565-585, 1969.Crossref 7. Parpart AK, Lorenz PB, Parpart ER, et al: The osmotic resistance of human red cells . J Clin Invest 26:636-640, 1947.Crossref 8. Weed RI, LaCelle PL, Merrill EW: Metabolic dependence of red cell deformability . J Clin Invest 48:795-809, 1969.Crossref 9. Gastel LFJ, van Steveninck J, van Bruijne AW: The influence of anesthetics on red cell deformability . Biochem Biophys Res Commun 55:1240-1245, 1973.Crossref 10. Knauf PA, Riordan JR, Schumann B, et al: Calcium-potassium-stimulated net potassium efflux from human erythrocyte ghosts . J Membr Biol 25:1-22, 1975.Crossref 11. Weed RL, Reed CF, Berg G: Is hemoglobin an essential structural component of human erythrocyte membranes? J Clin Invest 42:581-588, 1963.Crossref 12. Fairbanks G, Steck TL, Wallach DFH: Electrophoretic analysis of the major polypeptides of the human erythrocyte membrane . Biochemistry 10:2606-2617, 1971.Crossref 13. Kirkpatrick FH, Woods GM, LaCelle PL, et al: Calcium and magnesium ATPase of the spectrin fraction of human erythrocytes . J Supramol Struct 3:415-425, 1975.Crossref 14. Hosey MM, Tao M: Altered erythrocyte membrane phosphorylation in sickle cell disease . Nature 263:424-425, 1976.Crossref 15. Steck TL: The organization of proteins in the human red blood cell membrane . J Cell Biol 62:1-19, 1974.Crossref 16. Appel SH, Roses AD: Membrane biochemical studies in myotonic muscular dystrophy , in Bolis L, Hoffman JF, Leaf A (eds): Membranes and Diseases . New York, Raven Press, 1976, pp 183-195. 17. Brown HD, Chattpadhyay SK, Patel AB: Erythrocyte abnormality in human myopathy . Science 157:1577-1578, 1967.Crossref 18. Roses AD, Appel SH: Phosphorylation of component of the human erythrocyte membrane in myotonic muscular dystrophy . J Membr Biol 20:51-58, 1975.Crossref 19. Roses AD, Appel SH: Protein kinase activity in erythrocyte ghosts of patients with myotonic dystrophy . Proc Natl Acad Sci 70:1855-1859, 1973.Crossref 20. Butterfield DA, Chesnut DB, Roses AD, et al: Electron spin resonance studies in erythrocytes from patients with myotonic muscular dystrophy . Proc Natl Acad Sci 71:909-913, 1974.Crossref 21. Lassen UV, Pape L, Vestergaard-Bogind B: Effect of calcium on the membrane potential of amphiuma red cells . J Membr Biol 26:51-70, 1976.Crossref 22. Roses AD, Roses MJ, Miller SE, et al: Carrier detection in Duchenne muscular dystrophy . N Engl J Med 294:193-198, 1976.Crossref 23. Hoskins B, Maren TH, Vroom FQ, et al: Acetazolamide and potassium flux in red blood cells . Arch Neurol 31:187-191, 1974.Crossref 24. Vroom FQ, Jarrell MA, Maren TH: Acetazolamide treatment of hypokalaemic periodic paralysis . Arch Neurol 32:385-392, 1975.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Erythrocyte Membrane Studies in Familial Hypokalemic Periodic Paralysis

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1978.00500330063013
Publisher site
See Article on Publisher Site

Abstract

Abstract • A study of RBC membrane functions was performed in four patients suffering from familial hypokalemic periodic paralysis who had permanent muscular weakness. Electrophoretograms of membrane proteins, cell deformability, calciumpromoted potassium efflux, calcium-ATPase activity, and endogeneous phosphorylation of membrane proteins were all within the normal range. These results are compared with similar studies performed in myotonic and Duchenne-type dystrophies, in which abnormalities in the RBC membrane have been described. The results do not support the theory of RBC involvement in hypokalemic periodic paralysis. However, this does not imply that the muscle cell membrane is not involved in the underlying pathological process in this disorder. References 1. Oppenheim H: Neue Mitteilungen über den von Professor Westphal beschriebenen Fall von periodischer Lähmung aller vier Extremitäten . Charité Ann 16:350-372, 1891. 2. Engel AG, Lambert EH: Calcium activation of electrically inexcitable muscle fibers in primary hypokalaemic periodic paralysis . Neurology 19:851-858, 1969.Crossref 3. Bernhardt M: Notiz über die familiäre Form der Dystrophia muscularis progressiva und deren Combination mit periodisch auftretender paroxysmaler Lähmung . Dtsch Z Nervenheilk 8:111-118, 1895.Crossref 4. Rowland LP: Pathogenesis of muscular dystrophies . Arch Neurol 33:315-321, 1976.Crossref 5. Biemond A, Polak-Daniels A: Familial periodic paralysis and its transition into spinal muscular atrophy . Brain 57:91-108, 1934.Crossref 6. MacDonald RD, Rewcastle NB, Humphrey JG: Myopathy of hypokalaemic periodic paralysis . Arch Neurol 20:565-585, 1969.Crossref 7. Parpart AK, Lorenz PB, Parpart ER, et al: The osmotic resistance of human red cells . J Clin Invest 26:636-640, 1947.Crossref 8. Weed RI, LaCelle PL, Merrill EW: Metabolic dependence of red cell deformability . J Clin Invest 48:795-809, 1969.Crossref 9. Gastel LFJ, van Steveninck J, van Bruijne AW: The influence of anesthetics on red cell deformability . Biochem Biophys Res Commun 55:1240-1245, 1973.Crossref 10. Knauf PA, Riordan JR, Schumann B, et al: Calcium-potassium-stimulated net potassium efflux from human erythrocyte ghosts . J Membr Biol 25:1-22, 1975.Crossref 11. Weed RL, Reed CF, Berg G: Is hemoglobin an essential structural component of human erythrocyte membranes? J Clin Invest 42:581-588, 1963.Crossref 12. Fairbanks G, Steck TL, Wallach DFH: Electrophoretic analysis of the major polypeptides of the human erythrocyte membrane . Biochemistry 10:2606-2617, 1971.Crossref 13. Kirkpatrick FH, Woods GM, LaCelle PL, et al: Calcium and magnesium ATPase of the spectrin fraction of human erythrocytes . J Supramol Struct 3:415-425, 1975.Crossref 14. Hosey MM, Tao M: Altered erythrocyte membrane phosphorylation in sickle cell disease . Nature 263:424-425, 1976.Crossref 15. Steck TL: The organization of proteins in the human red blood cell membrane . J Cell Biol 62:1-19, 1974.Crossref 16. Appel SH, Roses AD: Membrane biochemical studies in myotonic muscular dystrophy , in Bolis L, Hoffman JF, Leaf A (eds): Membranes and Diseases . New York, Raven Press, 1976, pp 183-195. 17. Brown HD, Chattpadhyay SK, Patel AB: Erythrocyte abnormality in human myopathy . Science 157:1577-1578, 1967.Crossref 18. Roses AD, Appel SH: Phosphorylation of component of the human erythrocyte membrane in myotonic muscular dystrophy . J Membr Biol 20:51-58, 1975.Crossref 19. Roses AD, Appel SH: Protein kinase activity in erythrocyte ghosts of patients with myotonic dystrophy . Proc Natl Acad Sci 70:1855-1859, 1973.Crossref 20. Butterfield DA, Chesnut DB, Roses AD, et al: Electron spin resonance studies in erythrocytes from patients with myotonic muscular dystrophy . Proc Natl Acad Sci 71:909-913, 1974.Crossref 21. Lassen UV, Pape L, Vestergaard-Bogind B: Effect of calcium on the membrane potential of amphiuma red cells . J Membr Biol 26:51-70, 1976.Crossref 22. Roses AD, Roses MJ, Miller SE, et al: Carrier detection in Duchenne muscular dystrophy . N Engl J Med 294:193-198, 1976.Crossref 23. Hoskins B, Maren TH, Vroom FQ, et al: Acetazolamide and potassium flux in red blood cells . Arch Neurol 31:187-191, 1974.Crossref 24. Vroom FQ, Jarrell MA, Maren TH: Acetazolamide treatment of hypokalaemic periodic paralysis . Arch Neurol 32:385-392, 1975.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1978

References