Biomaterial Database

Abnormal phosphoenolpyruvate transport in erythrocytes of hereditary spherocytosis. 1981

H Ideguchi, and N Hamasaki, and Y Ikehara

The transport rates of phosphoenolpyruvate in erythrocytes from healthy individuals and from patients with hereditary spherocytosis were examined by incubating the cells with 10 mM phosphoenolpyruvate at 37 degrees C in the citrate buffer (0.10 M Na-citrate, 10 mM NaF, 5 mM glucose; pH 6.1 at 37 degrees C). The transport rate of phosphoenolpyruvate in erythrocytes of hereditary spherocytosis was 0.09 +/- 0.02 mumol/ml cells/min (mean +/- S. D., n = 7), whereas that in normal cells was 0.23 +/- 0.03 mumol/ml cells/min (mean +/- S. D., n = 7). The decreased rate of the transport seemed to be specific to the erythrocytes from patients with hereditary spherocytosis, since the rates in erythrocytes from patients with autoimmune hemolytic anemia, paroxysmal nocturnal hemoglobinuria, iron deficiency anemia and aplastic anemia were almost within normal range. Therefore, the decreased transport rate of phosphoenolpyruvate in erythrocytes of hereditary spherocytosis may be a reflection of some specific abnormality in the erythrocyte membrane. Moreover, the measurement of the transport rate of phosphoenolpyruvate may be used as a new diagnostic method for hereditary spherocytosis.

UI	MeSH Term	Description	Entries
D007700	Kinetics	The rate dynamics in chemical or physical systems.
D010728	Phosphoenolpyruvate	A monocarboxylic acid anion derived from selective deprotonation of the carboxy group of phosphoenolpyruvic acid. It is a metabolic intermediate in GLYCOLYSIS; GLUCONEOGENESIS; and other pathways.
D012016	Reference Values	The range or frequency distribution of a measurement in a population (of organisms, organs or things) that has not been selected for the presence of disease or abnormality.	Normal Range,Normal Values,Reference Ranges,Normal Ranges,Normal Value,Range, Normal,Range, Reference,Ranges, Normal,Ranges, Reference,Reference Range,Reference Value,Value, Normal,Value, Reference,Values, Normal,Values, Reference
D004912	Erythrocytes	Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.	Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000740	Anemia	A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.	Anemias
D001692	Biological Transport	The movement of materials (including biochemical substances and drugs) through a biological system at the cellular level. The transport can be across cell membranes and epithelial layers. It also can occur within intracellular compartments and extracellular compartments.	Transport, Biological,Biologic Transport,Transport, Biologic
D013103	Spherocytosis, Hereditary	A group of familial congenital hemolytic anemias characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions.	Hereditary Spherocytoses,Spherocytoses, Hereditary