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T Shimizu, N Kono, H Kiyokawa, Y Yamada, N Hara, I Mineo, M Kawachi, H Nakajima, YL Wang and S Tarui
Second Department of Internal Medicine, Osaka University Medical School,
Japan.
Levels of erythrocyte glycolytic intermediates after the
phosphofructokinase (PFK) step, including 2,3-bisphosphoglycerate (2,3-
DPG), were decreased at rest in patients from separate families with type
VII glycogenosis. The concentration of 2,3-DPG was about half of the normal
control value during a period of unrestricted daily activity but was
further decreased to one third of normal after a one-day bed rest. Mild
ergometric exercise rapidly increased the levels of fructose-
1,6-bisphosphate, dihydroxyacetone phosphate plus glyceraldehyde-3-
phosphate, and 2,3-DPG in patients' circulating erythrocytes but did not in
those of normal subjects. This indicated that a crossover point at the PFK
step in glycolysis disappeared after physical exercise and, consequently,
the 2,3-DPG concentration, which had decreased because of blockage of the
PFK step, was restored considerably. This apparently exercise-related
alteration in intermediary metabolism at the beginning of glycolysis was
reproduced in vitro by incubating normal erythrocytes in the presence of
inosine or ammonia, both of which have increased levels in circulating
blood during and after exercise in this disorder. We conclude that physical
activity in addition to a genetic deficiency in erythrocyte PFK affects
glycolysis in erythrocytes in type VII glycogenosis and that myogenic
factors released from exercising muscles may be responsible for this
change.
This article has been cited by other articles:
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| Copyright © 1988 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
