Redesign of EAF Peg Hb As an Oxygen Transfer Catalyst for Improved Tissue Oxygenation of Hypoxic Areas: A Therapeutic for Anemia and Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4845-4845
Author(s):  
Savita Bhutoria ◽  
Amy Tsai ◽  
Marcos Intaglietta ◽  
Sandra M. Suzuka ◽  
Sangeetha Thangaswamy ◽  
...  

Abstract Background Surface decoration of Hb with PEG chains has been advanced as an approach to attenuate the vasoconstrictive activity of acellular Hb in circulation. PEGylated Hbs with higher oxygen affinities relative to unmodified Hb has an advantage when designing blood substitutes in achieving targeted transport of O2 from lungs to tissues. Extension Arm Facilitated (EAF) PEGylation of Hb with six copies of PEG 5K (EAF P5K6 Hb) was advanced as an optimum level of PEGylation that does not induce weakening of interdimeric interactions in the quaternary structure of Hb. EAF P5K6 Hb at 4 gm % is an O2 carrying colloidal plasma expander. However, a prototype of EAF P5K6 Hb, developed by Sangart as MP4, did not increase microvascular oxygen delivery or improve tissue oxygenation in models of extreme hemodilution,whereas the EAF P5K6 Hb and P5K2 did better than the non-oxygen carrying semisynthetic colloidal plasma expander and EAF P5K6 albumin.Also, P5K2 Hb does a better tissue oxygenation even though its oxygen affinity is comparable to that of EAF P5K6 Hb1. As PEG chains increases from 2 to 10 copies, there is a lesser effect in the oxygen affinity of PEG Hbs in the presence of allosteric effectors like IHP and L-35. This suggests that higher degree of PEGylation preferentially stabilize the oxy conformation of PEG Hb, decreasing the efficacy of O2 release in the circulation. We attributed this decreased efficiency to the larger PEG shell which might lower the diffusion of O2out to the plasma. We reasoned that we could reduce the size of the PEG shell using six copies of PEG 3K and that we could use this new PEG Hb (EAF P3K6 Hb) at 6 gm% to get nearly the same viscosity as 4 gm % EAF P5K6 Hb. We compared tissue oxygenation by EAF P3K6 Hb with EAF P5K6 Hb and P5K2 Hb in an experimentally induced anemia model and tested its therapeutic efficacy in attenuating the experimentally induced acute crisis in a mild murine model of SCD, NY1DD. Methods EAF P3K6 Hb was prepared with PEG3K and characterized as EAF P5K6 Hb2. Molecular models and the shapes of PEG Hbs have been developed using InsightII program (Accelrys). Tissue oxygenation studies were carried out in the extreme hemodilution hamster model as described previously.1 Acute vaso-occlusion was studied using intravital microscopy in the venules of cremaster muscles of NY1DD SCD mice subjected to hypoxia-reoxygenation (18 hrs of hypoxia with 8% O2 and then normoxia).3The efficacy was measured after treatment with 10% top load of 6 gm % solution of EAF P3K6 Hb, given at the transitioning stage from hypoxia to normoxia Results PEG 3K chains reduced the molecular radius and volume while increased the packing density of PEG shell of EAF P3K6 Hb as compared to EAF P5K6 Hb (Table 1). The packing of the PEG chains induced a globular shape to the EAF P3K6 Hb vs ellipsoidal shape to EAF P5K6 Hb (Fig 1). Oxygen affinity of EAF P3K6 was comparable to that of EAF P5K6 Hb. In the extreme hemodilution hamster model of anemia, the efficacy of EAF P3K6 Hb to increase tissue oxygenation was comparable to that of P5K2 Hb (Fig 2). From the intravital micrographs of cremaster muscles (Fig 3), P3K6 Hb markedly attenuated the induced vaso-occlusive crisis in NY1DD mice. Discussion and Conclusions We have previously shown that EAF P3K6 Hb is effective in normalizing the chronic state of vaso-occlusion in BERK sickle mice. We demonstrate here that EAF P3K6 Hb protects NY1DD sickle mice from developing acute vaso-occlusion. These studies suggest that a smaller compact PEG shell around Hb increases the ability of the PEG Hb to oxygenate the tissues. NY1DD mice are not very anemic (Hb > 10 gm/dl); a 10% top load of 6 gm% high oxygen affinity Hb represents no significant increase in Hb level (≈0.06 gm %). We suggest that the ability of such a miniscule amount of PEG Hb in the plasma to protect the animal from hypoxia is due to its enhanced ability to deliver O2 under anemic and hypoxic conditions. We suggest that EAF P3K6 Hb facilitates improved extraction of O2 from RBC to plasma and to the subsequent diffusion to tissues, i.e. small amount of PEG Hb in plasma acts as a catalyst for O2 transfer while the oxyHb in RBC serves as the local reservoir for O2. This improved O2 extractionmay be effective in anemia in general, and have a unique application in sickle cell disease. References 1.Cabrales, P et al (2004) Am J Physiol Heart Circ Physiol, 287:H1609-H1617. 2. Acharya, S. A. et al (2007). Biochem J, 3.Kaul D.K. et al (2000) J Clin Invest, 106(5):715. Disclosures No relevant conflicts of interest to declare.

The Lancet ◽  
1986 ◽  
Vol 327 (8485) ◽  
pp. 831-834 ◽  
Author(s):  
A.J. Keidan ◽  
R.D. White ◽  
E.R. Huehns ◽  
I.M. Franklin ◽  
M. Joy ◽  
...  

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Paul Telfer ◽  
Irene Agodoa ◽  
Kathleen M. Fox ◽  
Laurie Burke ◽  
Timothy Mant ◽  
...  

For many patients with sickle cell disease (SCD), jaundice is a significant clinical disease manifestation that impacts on patient well-being. We report a case of a patient with SCD and chronic jaundice treated with voxelotor (GBT440), a novel small molecule hemoglobin oxygen affinity modulator and potential disease-modifying therapy for SCD. The case patient is a 27- year-old Black male with a long history of SCD with clinical jaundice and scleral icterus. After starting voxelotor, the patient reported that his jaundice cleared within one week, and that he felt much better with more energy, and was relieved after his eyes cleared. Voxelotor reduced bilirubin and unconjugated bilirubin (by up to 76%), and hemoglobin improved from 9.9 g/dL at baseline to 11.1 g/dL at 90 days. Jaundice impacts many adults with SCD, significantly impacting self-image. Voxelotor treatment reduced bilirubin levels and improved jaundice, resulting in an improved sense of well-being in our case patient.


Author(s):  
KRISHNA KUMAR ◽  
Nitish Kumar ◽  
Amresh gupta ◽  
Arpita singh ◽  
Pandey Swarnima ◽  
...  

Sickle cell anemia is a common disease in Oman country. In this disease, sickle-shaped cells are formed. These cells interrupt blood vessels and cause a reduction in oxygen transportation. It was founded that henna (Lawsonia inermis) can prohibit the formation of sickle cells. The Lawsone (2-Hydroxy-1,4-Naphthoquinone) is the constituents of henna which is responsible for the anti-sickling activity, by increasing the oxygen affinity of red blood cells. Hena has the anti-sickling activity which is proved by incubating aqueous and methanolic henna extracts with sickle cell disease patient's whole blood. Then for reduction to oxygen tension 2%, sodium bisulphite was added. Therefore, the percentage of sickled cells to normal red blood cells was observed at 30 minutes intervals. Henna proved a delay in the sickling process in 84% of the tested samples. Both extracts(aqueous and methanolic henna) can delay sickling for about an hour.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S100-S101
Author(s):  
S S Karimi ◽  
H Ni ◽  
L L Hsu

Abstract Introduction/Objective Voxelotor is a molecule that allosterically binds to the alpha-chain of hemoglobin, resulting in increased oxygen affinity. This allosteric inhibition leads to prevention of hemoglobin polymerization and sickling of red blood cells in response to low oxygen tension. Voxelotor has been used to treat patients with Sickle Cell Disease (SCD) and recent literature indicates it may contribute to complex hemoglobin fractionation (HF) elution patterns. We report a novel case of a SCD patient on concurrent Hydroxyurea, Voxelotor and chronic RBC exchange transfusion treatment and discuss the implications of these three treatment modalities on HF and monitoring of SCD. Methods A 17-year-old female with SCD complicated by frequent vaso-occlusive crisis, and avascular necrosis managed with chronic RBC exchange and Hydroxyurea. Her HF prior to initiation of Voxelotor treatment showed 3.2% HbA2, 51% HbA, 6.0% HbF, and 41% HbS. Voxelotor therapy was initiated at 1500mg/day and HF was performed 10 days later. Whole blood was collected and subjected to High Performance Liquid Chromatography (HPLC) with reflex to RBC solubility and Capillary Electrophoresis. Results HF performed post-Voxelotor therapy revealed positive sickle solubility with a complex pattern of 2.7% HbA2, 49.2% HbA, 5.3% HbF, 15.7% HbS, 0% HbC, and two additional peaks of a 6.3% peak in the window-D region (retention time of 4.34) and 20.8% of an atypical Hb peak pattern (at the retentuin time of 4.18). The results reflected a complex HF of a HbSS patient on concurrent chronic RBC exchange transfusion, hydroxyurea therapy, and Voxelotor treatment. Post Voxelotor-therapy HF revealed a reduction in HbS from 41% to 15.7% with the emergence of two additional peaks. Chronic RBC exchange transfusion and Hydroxyurea treatment account for the observed fractionation of HbA and HbF, respectively. Based on recent literature, we attribute the emergence of the two additional peaks to Voxelotor therapy. All three therapies led to reduction in HbS. Conclusion Routine HF serves as an essential modality in diagnosis and monitoring of SCD. Voxelotor treatment alters the HF profile and may cause difficulty for interpretation. With the emergence of novel therapies, it is imperative for clinicians to provide medication information to clinical laboratories and pathologists to be fully aware of the effects of current treatments to correctly interpret and monitor SCD.


2017 ◽  
Vol 114 (5) ◽  
pp. E689-E696 ◽  
Author(s):  
Quan Li ◽  
Eric R. Henry ◽  
James Hofrichter ◽  
Jeffrey F. Smith ◽  
Troy Cellmer ◽  
...  

Although it has been known for more than 60 years that the cause of sickle cell disease is polymerization of a hemoglobin mutant, hydroxyurea is the only drug approved for treatment by the US Food and Drug Administration. This drug, however, is only partially successful, and the discovery of additional drugs that inhibit fiber formation has been hampered by the lack of a sensitive and quantitative cellular assay. Here, we describe such a method in a 96-well plate format that is based on laser-induced polymerization in sickle trait cells and robust, automated image analysis to detect the precise time at which fibers distort (“sickle”) the cells. With this kinetic method, we show that small increases in cell volume to reduce the hemoglobin concentration can result in therapeutic increases in the delay time prior to fiber formation. We also show that, of the two drugs (AES103 and GBT440) in clinical trials that inhibit polymerization by increasing oxygen affinity, one of them (GBT440) also inhibits sickling in the absence of oxygen by two additional mechanisms.


Blood ◽  
1994 ◽  
Vol 84 (9) ◽  
pp. 3189-3197 ◽  
Author(s):  
M Trudel ◽  
ME De Paepe ◽  
N Chretien ◽  
N Saadane ◽  
J Jacmain ◽  
...  

Erythrocyte sickling on deoxygenation in vitro occurs in transgenic SAD mice, hemizygous for a modified human sickle hemoglobin, HbSAD [alpha 2 beta 2S(beta 6val)Antilles (beta 23 lle)D- Punjab (beta 121Gln)] (SAD- 1, 19% HbSAD; beta-thal/SAD-1, 26% HbSAD). The present study examines the cellular defects in vivo and pathologic changes observed in SAD-1 mice at atmospheric oxygenation as well as the effect of acute hypoxia. The transgenic mice showed generalized congestion and microvascular occlusions, occasionally with thrombosis and infarctions of lung, kidneys, penis, and myocardium. The most prevalent chronic organ lesions were congestive splenomegaly (83% of animals) and renal glomerulopathy, which affected 75% of animals by 10 months of age. Further, SAD mice have a mean lifespan that was reduced by 40% when compared with nontransgenic littermates. Premature death of SAD mice was associated with acute vasoocclusive events or severe renal disease. SAD mice developed lethal vasoocclusive processes when exposed to reduced pO2 conditions, whereas control mice survived normally. The sensitivity to hypoxia appears to depend on the cellular level of HbSAD, because death occurred at pO2 of 42 mmHg for SAD mice and 49 mmHg for beta-thal/SAD. Administration of an antisickling agent that increases oxygen affinity (BW12C79) protected SAD and beta-thal/SAD mice from the lethal hypoxic stress. In conclusion, the transgenic SAD and beta-thal/SAD mice developed a pathophysiology that strongly resembles human sickle cell disease. Moreover, this animal model allows studies on the effect of antisickling agents.


Blood ◽  
2021 ◽  
Author(s):  
Eric R Henry ◽  
Belhu Metaferia ◽  
Quan Li ◽  
Julia Harper ◽  
Robert B Best ◽  
...  

The issue of treating sickle cell disease with drugs that increase hemoglobin oxygen affinity has come to the fore with the FDA approval in 2019 of voxelotor, the only anti-sickling drug approved since hydroxyurea in 1998. Voxelotor reduces sickling by increasing the concentration of the non-polymerizing, high oxygen affinity R (oxy) conformation of HbS. Treatment of sickle cell patients with voxelotor increases Hb levels and decreases indicators of hemolysis, but with no indication as yet that it reduces the frequency of pain episodes. Here we use the allosteric model of Monod, Wyman, and Changeux to simulate whole blood oxygen dissociation curves and red cell sickling in the absence and presence of voxelotor under the in vivo conditions of rapid oxygen pressure decreases. Our modeling agrees with experiments using a new robust assay, which shows the very large, expected decrease in sickling from the drug. The modeling indicates, however, that the increase in oxygen delivery from reduced sickling is largely offset by the increase in oxygen affinity. The net result is that the drug increases overall oxygen delivery only at the very lowest oxygen pressures. Reduction of sickling does, however, mitigate against red cell damage and explains the observed decrease in hemolysis. More importantly, our modeling of in vivo oxygen dissociation, sickling, and oxygen delivery suggests that drugs that increase fetal hemoglobin or decrease MCHC, should be more therapeutically effective than drugs that increase oxygen affinity.


MedChemComm ◽  
2019 ◽  
Vol 10 (11) ◽  
pp. 1900-1906 ◽  
Author(s):  
A. M. Omar ◽  
T. David ◽  
P. P. Pagare ◽  
M. S. Ghatge ◽  
Q. Chen ◽  
...  

The intracellular polymerization and the concomitant sickling processes, central to the pathology of sickle cell disease, can be mitigated by increasing the oxygen affinity of sickle hemoglobin (HbS).


1989 ◽  
Vol 76 (4) ◽  
pp. 357-362 ◽  
Author(s):  
A. J. Keidan ◽  
M. C. Sowter ◽  
C. S. Johnson ◽  
S. S. Marwah ◽  
J. Stuart

1. The formation of polymerized haemoglobin S in sickle cells is critically dependent on the concentration of deoxygenated haemoglobin so that compounds which increase the oxygen affinity of haemoglobin S are potential anti-sickling agents. 2. BW12C [5-(2-formyl-3-hydroxyphenoxy)pentanoic acid] and BWA589C [4-(2-formyl-3-hydroxyphenoxymethyl)benzoic acid] are aromatic benzaldehydes that cause a dose-dependent left-shift of the oxygen saturation curve of haemoglobin S by stabilization of its oxy-(R)-conformation. 3. A 5 μm pore filtration method, which is highly sensitive to polymerization of haemoglobin S, was used to demonstrate a significant improvement in the deformability of deoxygenated sickle erythrocytes at concentrations (0.75–1.5 mmol/l) of BW12C and BWA589C that are achievable in vivo. Both compounds may therefore be of value for the treatment of sickle cell disease. 4. Filtration of sickle cells through pores of 5μm diameter is a sensitive technique for evaluating the rheological effects of potential anti-sickling compounds.


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