scholarly journals Generation and Export of Red Blood Cell ATP in Health and Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Timothy J. McMahon ◽  
Cole C. Darrow ◽  
Brooke A. Hoehn ◽  
Hongmei Zhu
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Dynamic Control ◽  
Nicotinamide Adenine Dinucleotide Phosphate ◽  
Antioxidant Systems ◽  
Atp Production ◽  
Allosteric Effector ◽  
The Face ◽  
Differential Control ◽  
Glucose 6 Phosphate Dehydrogenase

Metabolic homeostasis in animals depends critically on evolved mechanisms by which red blood cell (RBC) hemoglobin (Hb) senses oxygen (O2) need and responds accordingly. The entwined regulation of ATP production and antioxidant systems within the RBC also exploits Hb-based O2-sensitivity to respond to various physiologic and pathophysiologic stresses. O2 offloading, for example, promotes glycolysis in order to generate both 2,3-DPG (a negative allosteric effector of Hb O2 binding) and ATP. Alternatively, generation of the nicotinamide adenine dinucleotide phosphate (NADPH) critical for reducing systems is favored under the oxidizing conditions of O2 abundance. Dynamic control of ATP not only ensures the functional activity of ion pumps and cellular flexibility, but also contributes to the availability of vasoregulatory ATP that can be exported when necessary, for example in hypoxia or upon RBC deformation in microvessels. RBC ATP export in response to hypoxia or deformation dilates blood vessels in order to promote efficient O2 delivery. The ability of RBCs to adapt to the metabolic environment via differential control of these metabolites is impaired in the face of enzymopathies [pyruvate kinase deficiency; glucose-6-phosphate dehydrogenase (G6PD) deficiency], blood banking, diabetes mellitus, COVID-19 or sepsis, and sickle cell disease. The emerging availability of therapies capable of augmenting RBC ATP, including newly established uses of allosteric effectors and metabolite-specific additive solutions for RBC transfusates, raises the prospect of clinical interventions to optimize or correct RBC function via these metabolite delivery mechanisms.

A proposal for further standardization of red blood cell glucose 6-phosphate dehydrogenase determinations

1988 ◽  
Vol 10 (4) ◽  
pp. 409-416
Author(s):  
J.C. ARDERN ◽  
NORMA EDWARDS ◽  
K. HYDE ◽  
CARMEN JARDINE-WILKINSON ◽  
KORNELIA I. CINKOTAI ◽  
...  
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Cell Glucose

scholarly journals Contribution of the band 3-ankyrin interaction to erythrocyte membrane mechanical stability

Blood ◽  
1991 ◽  
Vol 77 (7) ◽  
pp. 1581-1586 ◽  
Author(s):  
PS Low ◽  
BM Willardson ◽  
N Mohandas ◽  
M Rossi ◽  
S Shohet
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Mechanical Stability ◽  
Membrane Integrity ◽  
Membrane Skeleton ◽  
Band 3 ◽  
Cell Membrane Integrity ◽  
The Face ◽  
Cell Stability

Abstract In an effort to evaluate the role of the band 3-ankyrin linkage in maintenance of red blood cell membrane integrity, solution conditions were sought that would selectively dissociate the band 3-ankyrin linkage, leaving other membrane skeletal interactions intact. For this purpose erythrocytes were equilibrated overnight in nutrient-containing buffers at a range of elevated pHs and then examined for changes in mechanical stability and membrane skeletal composition. Band 3 was found to be released from interaction with the membrane skeleton over a pH range (8.4 to 9.5) that was observed to dissociate the band 3- ankyrin interaction in vitro. In contrast, all other membrane skeletal associations appeared to remain intact up to pH 9.3, after which they were also seen to dissociate. Whereas hemolysis of mechanically unstressed cells did not begin until approximately pH 9.3, where the membrane skeletons began to disintegrate, enhanced fragmentation of shear stressed membranes was seen to begin near pH 8, where band 3 dissociation was first observed. Furthermore, the shear-induced fragmentation rate was found to reach a maximum at pH 9.4, ie, where band 3 dissociation was essentially complete. Based on these correlations, we hypothesize that the band 3-ankyrin linkage of the membrane skeleton to the lipid bilayer is essential for red blood cell stability in the face of mechanical distortion but not for cellular integrity in the absence of mechanical stress.

scholarly journals Some Properties of Red Blood Cell Glucose-6-Phosphate Dehydrogenase from three Species of Kangaroo

1970 ◽  
Vol 23 (5) ◽  
pp. 1305 ◽  
Author(s):  
BJ Richardson ◽  
AB Czuppon
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Coastal Areas ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Cell Glucose

Richardson and Czuppon (1969) reported a cline in red blood cell glucose-6- phosphate dehydrogenase levels in a marsupial-the wallaroo Macropu8 robustu8 Gould, 1841. High dehydrogenase levels were found in animals from the dry inland areas of Australia, while animals from the wetter eastern coastal areas had lower levels.

scholarly journals Diagnosis and clinical management of enzymopathies

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 341-352
Author(s):  
Lucio Luzzatto
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Iron Chelation ◽  
Hematopoietic Stem ◽  
Dark Urine ◽  
Mild Anemia ◽  
Cell Enzyme ◽  
Genetically Determined ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Chronic Hemolytic Anemia

Abstract At least 16 genetically determined conditions qualify as red blood cell enzymopathies. They range in frequency from ultrarare to rare, with the exception of glucose-6-phosphate dehydrogenase deficiency, which is very common. Nearly all these enzymopathies manifest as chronic hemolytic anemias, with an onset often in the neonatal period. The diagnosis can be quite easy, such as when a child presents with dark urine after eating fava beans, or it can be quite difficult, such as when an adult presents with mild anemia and gallstones. In general, 4 steps are recommended: (1) recognizing chronic hemolytic anemia; (2) excluding acquired causes; (3) excluding hemoglobinopathies and membranopathies; (4) pinpointing which red blood cell enzyme is deficient. Step 4 requires 1 or many enzyme assays; alternatively, DNA testing against an appropriate gene panel can combine steps 3 and 4. Most patients with a red blood cell enzymopathy can be managed by good supportive care, including blood transfusion, iron chelation when necessary, and splenectomy in selected cases; however, some patients have serious extraerythrocytic manifestations that are difficult to manage. In the absence of these, red blood cell enzymopathies are in principle amenable to hematopoietic stem cell transplantation and gene therapy/gene editing.

Microvascular responses to hemodilution with Hb vesicles as red blood cell substitutes: influence of O2 affinity

1999 ◽  
Vol 276 (2) ◽  
pp. H553-H562 ◽  
Author(s):  
Hiromi Sakai ◽  
Amy G. Tsai ◽  
Ronald J. Rohlfs ◽  
Hiroyuki Hara ◽  
Shinji Takeoka ◽  
...  
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Carrying Capacity ◽  
Capillary Density ◽  
Microvascular Perfusion ◽  
Phospholipid Vesicles ◽  
Molar Ratios ◽  
Allosteric Effector ◽  
Syrian Golden Hamsters ◽  
Optimal Value

Phospholipid vesicles encapsulating purified hemoglobin (HbV) were developed to provide O2-carrying capacity to plasma expanders. Microvascular perfusion was determined for HbV with different O2 affinity (P50 = 9, 16, and 30 mmHg) prepared by coencapsulating pyridoxal 5′-phosphate (PLP) at the molar ratios of [PLP]/[Hb] = 0, 0.5, and 3, respectively (cf. hamster blood, P50: 28 mmHg), and suspended in 8 g/dl human serum albumin (HSA). Eighty percent of the red blood cell (RBC) mass of conscious Syrian golden hamsters fitted with dorsal skinfold windows was substituted with either of the HbV-HSA suspensions, washed hamster RBC suspended in HSA (RBC-HSA), and HSA alone. All three HbV-HSA groups and RBC-HSA groups showed stable blood pressure and heart rate, which could not be sustained with HSA alone. Only the HbV (P50 = 9)-HSA group showed an increase in arterial O2tension (89.8 ± 14.7 mmHg, baseline 58.4 ± 4.0 mmHg) because of hyperventilation, and microvascular perfusion was decreased, indicating that facilitated O2 unloading of HbV by decreasing the O2 affinity (increasing P50) with PLP as an allosteric effector is important. Microvascular perfusion and microvascular and interstitial O2tensions in the HbV (P50 = 16 and 30)-HSA groups were significantly higher than those in the HSA group. The O2 release rate from the HbV was 18–32 s−1 vs. 4.4 s−1 for RBC. Functional capillary density was improved from 17 to 41% on average by decreasing P50 from 30 to 16 mmHg, which appears to be an optimal value for the P50 in this system.

Red Blood Cell Hexokinase in Tumor Bearing Mice

1983 ◽  
Vol 69 (3) ◽  
pp. 201-204 ◽  
Author(s):  
Mauro Magnani ◽  
Sabina Bonfigli ◽  
Franco Canestrari ◽  
Vilberto Stocchi ◽  
Marina Dachà ◽  
...  
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Red Cell ◽  
Normal Range ◽  
Tumor Bearing ◽  
Cell Age ◽  
Red Cell Enzymes ◽  
Isozymic Pattern ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Normal Controls

Red blood cell hexokinase of tumor-bearing BALB/c mice was found to be 35% higher than in the normal controls, whereas glucose 6-phosphate-dehydrogenase and other red blood cell glycolytic enzymes were in the normal range. This hexokinase increase cannot be explained by a mean younger red cell population because normal hematological data and normal red cell enzymes, known as red cell age-markers, have been found in tumor-bearing mice. The isozymic pattern of red cell hexokinase is not modified in the tumor-bearing mice.

scholarly journals Interpopulation Differences in Red Blood Cell Enzyme Levels of the Wallaroo Macropus Robustus (Marsupialia)

1970 ◽  
Vol 23 (3) ◽  
pp. 617 ◽  
Author(s):  
BJ Richardson ◽  
AB Czuppon
Keyword(s):  
Blood Cell ◽  
Substrate Specificity ◽  
Red Blood Cell ◽  
Glycolytic Enzymes ◽  
Cell Enzyme ◽  
Different Populations ◽  
Glucose 6 Phosphate Dehydrogenase

The levels of six glycolytic enzymes were studied in three different populations of M. robU8tU8. Significant interpopulation differences were found for five of the enzymes. Glucose-6-phosphate dehydrogenase levels are up to three times higher than those found in eutherians. Keto-I-phospha.te aldolase substrate specificity differs radically from that found in eutherians. The enzymes studied are independently regulated.

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