The activity of sorbitol dehydrogenase in some mammalian erythrocytes.
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Biomedical subjects
Publications and source records attributed to N S Agar.
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1. Activities of several enzymes associated with glucose metabolism were measured in the red blood cells of Saanen and Angora breed of goat. 2. No significant breed differences were observed in any enzyme activity.
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A comparative study of the red cell characters, glycolytic enzymes and intermediates was made in the domestic dog and the dingo. No significant differences were found in any of the parameters studied except the enzyme level of NADH-MR which was significantly lower in dingoes (P less than 0.05).
1. The levels of reduced glutathione (GSH) were measured in the red blood cells of 259 cattle of two diverse populations consisting of various breeds. 2. There was a uniform distribution of GSH values ranging from 40.0 to 113.0 mg/100 ml red cells with a mean value of 72.0 mg +/- 1.26 (S.E.). 3. An absence of bimodal distribution was, thus, shown in the cattle breeds examined.
Two male subjects are described, with unusual clinical presentations and with hitherto undescribed G6PD variants. The first, of Italian extraction, suffered from severe neonatal jaundice following maternal ingestion of fresh broad beans (Vicia fava) both prenatally and postnatally: the expression of the enzymatic defect was much more severe in the neonatal period than on retesting in adolescence, when biochemical characterization showed unique features which justify designation as a new variant Gd(+) Alexandra. The second patient, a boy of Maltese extraction who was found to have bilateral lamellar cataracts at the age of 4 years, was identified as G6PD deficient only as a result of a survey of children of Mediterranean origin with unexplained cataract formation; he has approximately 15% of normal enzyme activity, with another unique combination of biochemical characteristics which has led to its designation as Gd(-) Camperdown. Although this association may be coincidental, it prompts further attention to the possibility that under certain circumstances G6PD deficiency may favor cataract formation. The two cases illustrate the value of characterization of the mutant enzyme whenever unexpected clinical or laboratory results are obtained.
Glucose utilization, lactate production and glutathione regeneration were measured in the red blood cells of 2 species of Australian Marsupials, Eastern grey Kangaroo (Macropus gigantus) and red kangaroo (Macropus rufus), and were found to be significantly lower in the red blood cells from grey than that of red kangaroos.
A case is described of mild haemolytic anaemia in a female homozygous for pyruvate kinase deficiency, with 20% of normal enzyme activity and with the unexplained associations that she suffers from angina pectoris and that three of her children died in childhood from a muscular dystrophy presumed to be Werdnig-Hoffman disease.
Based on the observations made on human and dog red blood cells, it was recently proposed that the response of red cell glycolysis to variations in the levels of specific cations in an evolutionary adaptation in response to a specific cellular environment. We have now examined the effect of K+ and Na+ on the activity of pyruvate kinase (PK) in the red blood cells from 2 genetically different types of sheep high potassium (HK) and low potassium (LK). The results indicate that K+ stimulate glucose consumption and the activity of PK in both types of sheep. It thus appears that red cell PK from LK sheep does not fit into the concept of cellular environment and PK activity.
There was no significant difference in the level of 2,3-DPG in the red blood cells of sheep of different haemoglobin types (Hb A and Hb B) or potassium types (HK and LK). However, low glutathione (GSHL) sheep had significantly higher (p less than 0.01) level of 2,3-DPG in their red blood cells than high glutathione (GSHH) sheep. There was also significant effect of interactions between glutathione, haemoglobin and potassium types (p less than 0.05) and glutathione and haemoglobin types (p less than 0.01) on red cell 2,3-DPG levels.
Experimental anaemia resulted in an increase of red cell 2,3-DPG from 0.11 micronM/g Hb to 0.99 micronM in haemoglobin A sheep and from 0.21 micronM in haemoglobin B sheep. Production of haemoblobin Cas a result of anaemia was confined to haemoblobin A only. The results, therefore, appear to suggest that the rise in 2,3-DPG in the red blood cells of different haemoblobin types is independent of haemoblobin C.
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The erythrocytes of newborn and adult goats were studied with respect to GSH stability, GSH regeneration, levels of ATP and 2, 3-DPG, glucose consumption and activities of nine different enzymes of Embden-Meyerhof and pentose phosphate pathways of glucose metabolism. Red cells from newborn goats had significantly higher levels of ATP and 2, 3-DPG. The activities of all the enzymes measured were also significantly greater in the newborn. It is suggested that newborn goats possess red blood cells that are metabolically more active than those of adult goats.
The affinity of hemoglobin for oxygen did not differ significantly between normal and glutathione-deficient sheep. Although phlebotomy in sheep of both types did not significantly alter the partial pressure values at which hemoglobin is 50% saturated with oxygen, it did increase significantly the erythrocyte glutathione and 2,3-diphosphoglycerate (GSH) and (DPG) concentrations. Because DPG does not bind to sheep hemoglobin, its increase in hypoxic sheep must mean DPG is metabolically controlled in the absence of physiologic effect.
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