scholarly journals Erythrocyte glycolysis and its marked alterations by muscular exercise in type VII glycogenosis

Blood ◽  
1988 ◽  
Vol 71 (4) ◽  
pp. 1130-1134 ◽  
Author(s):  
T Shimizu ◽  
N Kono ◽  
H Kiyokawa ◽  
Y Yamada ◽  
N Hara ◽  
...  

Abstract 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.

Blood ◽  
1988 ◽  
Vol 71 (4) ◽  
pp. 1130-1134
Author(s):  
T Shimizu ◽  
N Kono ◽  
H Kiyokawa ◽  
Y Yamada ◽  
N Hara ◽  
...  

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.


1995 ◽  
Vol 7 (1) ◽  
pp. 107 ◽  
Author(s):  
LM Porter ◽  
AR Jones

In the presence of 3-bromo-1-hydroxypropanone (BOP), cauda epididymal sperm obtained from mature boars produced a carbonyl compound which is assumed to be (S)-3-bromolactaldehyde. Glyceraldehyde 3-phosphate dehydrogenase was rapidly inhibited which resulted in the accumulation of dihydroxyacetone phosphate and fructose-1,6-bisphosphate, and no accumulation of lactate when fructose was the substrate. The energy charge potential of the cells declined in the presence of BOP when either fructose or glycerol were substrates. It is suggested that BOP is transformed into (S)-3-bromolactaldehyde, which is the actual inhibitor of glyceraldehyde 3-phosphate dehydrogenase, thus demonstrating BOP to be the first brominated chemical to have an anti-glycolytic action on mature sperm in vitro.


1974 ◽  
Vol 47 (6) ◽  
pp. 577-588 ◽  
Author(s):  
L. J. Fairweather ◽  
J. Walker ◽  
D. C. Flenley

1. The position of the oxygen-haemoglobin dissociation curve has been related to erythrocyte 2,3-diphosphoglycerate (2,3-DPG) concentrations in twenty-seven patients with both acute and chronic respiratory failure. 2. The average values of both 2,3-DPG concentration and of the oxygen tension for one-half full saturation (P50) in these patients were normal, but values both above and below the normal range were encountered. 3. P50 values correlated positively with erythrocyte 2,3-DPG concentrations, but negatively correlated with the mean corpuscular haemoglobin concentration in these patients. 4. Multiple regression, including the above two variables and also the blood oxygen capacity as major factors, accounted for just under half of the variance in the position of the dissociation curve, and this was little improved by addition of the minor effects of the arterial oxygen and carbon dioxide tensions. 5. The intraerythrocytic pH bore a similar relationship to plasma pH, in both studies in vitro of blood from normal subjects and patients with chronic hypercapnia, and studies in vivo in hypercapnic subjects.


1979 ◽  
Vol 47 (3) ◽  
pp. 478-481 ◽  
Author(s):  
H. H. Kerr ◽  
G. A. Pantely ◽  
J. Metcalfe ◽  
J. E. Welch

Human blood oxygen affinity (BOA) was measured after blood from six normal donors was incubated with 4 concentrations of dihydroxyacetone (0.022, 0.044, 0.088, and 0.175 M) plus equimolar disodium phosphate and pyruvate (sodium salt) (0.013, 0.025, 0.05 and 0.1 M) in solutions labeled DDP X 1, DDP X 2, DDP X 4, and DDP X 8, respectively. Blood P50 rose (BOA was reduced) from a control value of 26.0 +/- 0.4 Torr (mean +/- SD) to 29.4 +/- 0.6, 30.6 +/- 0.4, 31.9 +/- 0.15 and 33.3 +/- 1.4 Torr after 2 h of incubation at 37 degrees C with solutions DDP X 1, DDP X 2, DDP X 4, and DDP X 8, respectively. P50 changes at 2 h were 75% complete within 30 min. During these incubations, erythrocyte 2,3-diphosphoglycerate (2,3-DPG) concentration rose from 0.76 +/- 0.09 mol/mol Hb (control) to 1.09 +/- 0.17, 1.14 +/- 0.10, 1.33 +/- 0.15, and 1.45 +/- 0.25 mol/mol Hb with increasing solution concentration. BOA is decreased by an increase in erythrocyte 2,3-DPG. Reduced BOA may improve oxygen delivery to ischemic tissues.


1962 ◽  
Vol 02 (02) ◽  
pp. 165-172
Author(s):  
C Miras ◽  
G Lewis ◽  
J Mantzos

Summary1. Separated leukocytes or total blood from normal subjects, untreated leukaemic patients and from leukaemic patients treated with cytostatic agents were incubated with CH3COONa-l-C14. Radioactivity of mixed lipids was measured at standard time intervals.2. The time incorporation curve observed with leukocytes from treated leukaemic patients showed after an initial linear part, a more rapid levelling off than the curves observed with leukocytes from untreated and normal subjects.3. Therefore, an indirect effect of treatment on leukocyte lipid synthesis seems to be present.4. Phospholipid and neutral lipid synthesis by leukaemic leukocytes was also studied. The results give no evidence that these fractions as a whole have any precursor-product relation.


1991 ◽  
Vol 30 (01) ◽  
pp. 35-39 ◽  
Author(s):  
H. S. Durak ◽  
M. Kitapgi ◽  
B. E. Caner ◽  
R. Senekowitsch ◽  
M. T. Ercan

Vitamin K4 was labelled with 99mTc with an efficiency higher than 97%. The compound was stable up to 24 h at room temperature, and its biodistribution in NMRI mice indicated its in vivo stability. Blood radioactivity levels were high over a wide range. 10% of the injected activity remained in blood after 24 h. Excretion was mostly via kidneys. Only the liver and kidneys concentrated appreciable amounts of radioactivity. Testis/soft tissue ratios were 1.4 and 1.57 at 6 and 24 h, respectively. Testis/blood ratios were lower than 1. In vitro studies with mouse blood indicated that 33.9 ±9.6% of the radioactivity was associated with RBCs; it was washed out almost completely with saline. Protein binding was 28.7 ±6.3% as determined by TCA precipitation. Blood clearance of 99mTc-l<4 in normal subjects showed a slow decrease of radioactivity, reaching a plateau after 16 h at 20% of the injected activity. In scintigraphic images in men the testes could be well visualized. The right/left testis ratio was 1.08 ±0.13. Testis/soft tissue and testis/blood activity ratios were highest at 3 h. These ratios were higher than those obtained with pertechnetate at 20 min post injection.99mTc-l<4 appears to be a promising radiopharmaceutical for the scintigraphic visualization of testes.


1988 ◽  
Vol 60 (02) ◽  
pp. 205-208 ◽  
Author(s):  
Paul A Kyrle ◽  
Felix Stockenhuber ◽  
Brigitte Brenner ◽  
Heinz Gössinger ◽  
Christian Korninger ◽  
...  

SummaryThe formation of prostacyclin (PGI2) and thromboxane A2 and the release of beta-thromboglobulin (beta-TG) at the site of platelet-vessel wall interaction, i.e. in blood emerging from a standardized injury of the micro vasculature made to determine bleeding time, was studied in patients with end-stage chronic renal failure undergoing regular haemodialysis and in normal subjects. In the uraemic patients, levels of 6-keto-prostaglandin F1α (6-keto-PGF1α) were 1.3-fold to 6.3-fold higher than the corresponding values in the control subjects indicating an increased PGI2 formation in chronic uraemia. Formation of thromboxane B2 (TxB2) at the site of plug formation in vivo and during whole blood clotting in vitro was similar in the uraemic subjects and in the normals excluding a major defect in platelet prostaglandin metabolism in chronic renal failure. Significantly smaller amounts of beta-TG were found in blood obtained from the site of vascular injury as well as after in vitro blood clotting in patients with chronic renal failure indicating an impairment of the a-granule release in chronic uraemia. We therefore conclude that the haemorrhagic diathesis commonly seen in patients with chronic renal failure is - at least partially - due to an acquired defect of the platelet a-granule release and an increased generation of PGI2 in the micro vasculature.


1990 ◽  
Vol 64 (03) ◽  
pp. 402-406 ◽  
Author(s):  
M D Oethinger ◽  
E Seifried

SummaryThe present in vitro study investigated dose-, time- and temperature-dependent effects of two-chain urokinase plasminogen activato(u-PA, urokinase) on normal citrated plasma. When 10 μg/ml u-PA wereadded to pooled normal plasma and incubated for 30 min at an ambient temperature (25° C), α2-antiplas-min decreased to 8% of the control value. Incubation on ice yielded a decrease to 45% of control,whereas α2-antiplasmin was fully consumed at 37° C. Fibrinogen and plasminogen fell to 46% and 39%, respectively, after a 30 min incubation at 25° C. Thrombin time prolonged to 190% of control.Various inhibitors were studied with respect to their suitability and efficacy to prevent these in vitro effects. Aprotinin exhibited a good protective effect on fibrinogen at concentrations exceeding 500 KlU/ml plasma. Its use, however, was limited due to interferences with some haemostatic assays. We could demonstrate that L-Glutamyl-L-Glycyl-L-Arginyl chloromethyl ketone (GGACK) and a specific polyclonal anti-u-PA-antibody (anti-u-PA-IgG) effectively inhibited urokinase-induced plasmin generation without interfering with haemostatic assays. The anti-u-PA-antibody afforded full protection ofα2-antiplasmin at therapeutic levels of u-PA.It is concluded that u-PA in plasma samples from patients during thrombolytic therapy may induce in vitro effects which should be prevented by the use of a suitable inhibitor such as GGACK or specific anti-u-PA-antibody.


1987 ◽  
Vol 58 (03) ◽  
pp. 921-926 ◽  
Author(s):  
E Seifried ◽  
P Tanswell

SummaryIn vitro, concentration-dependent effects of rt-PA on a range of coagulation and fibrinolytic assays in thawed plasma samples were investigated. In absence of a fibrinolytic inhibitor, 2 μg rt-PA/ml blood (3.4 μg/ml plasma) caused prolongation of clotting time assays and decreases of plasminogen (to 44% of the control value), fibrinogen (to 27%), α2-antiplasmin (to 5%), FV (to 67%), FVIII (to 41%) and FXIII (to 16%).Of three inhibitors tested, a specific polyclonal anti-rt-PA antibody prevented interferences in all fibrinolytic and most clotting assays. D-Phe-Pro-Arg-CH2Cl (PPACK) enabled correct assays of fibrinogen and fibrinolytic parameters but interfered with coagulometric assays dependent on endogenous thrombin generation. Aprotinin was suitable only for a restricted range of both assay types.Most in vitro effects were observed only with rt-PA plasma concentrations in excess of therapeutic values. Nevertheless it is concluded that for clinical application, collection of blood samples on either specific antibody or PPACK is essential for a correct assessment of in vivo effects of rt-PA on the haemostatic system in patients undergoing fibrinolytic therapy.


1968 ◽  
Vol 57 (1) ◽  
pp. 23-32 ◽  
Author(s):  
Hironori Nakajima ◽  
Mitsunori Murala ◽  
Masumitsu Nakata ◽  
Takeshi Naruse ◽  
Seiji Kubo

ABSTRACT The in vitro resin uptake of 3H-prednisolone was used for the determination of blood cortisol after addition of radioactive prednisolone followed by Amberlite CG 400 Type 1 to the test serum, and incubation of the mixture. The radioactivity of the supernatant was compared before and after the addition of the resin. The principle of this method is similar to that of the 131I-triiodothyronine resin uptake for the thyroid function test. The tests for the specificity, reproducibility and sensitivity gave satisfactory results. The mean basal value ± SD of the 3H-prednisolone resin uptake was 35.3 ± 9.2% in normal subjects, and 27.1 ± 4.8% in pregnant women. This method was valid in various adrenal function tests, i. e. the adrenal circadian rhythm, corticotrophin (ACTH) test, dexamethasone suppression test and the adrenal response to lysine-8-vasopressin. It proved to be a sensitive indicator of the adrenal function. These results suggest that this method should be useful for a routine adrenal function test.


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