Abdulmalik O, Ghatge MS, Musayev FN, Parikh A, Chen Q, Yang J, Nnamani I, Danso-Danquah R, Eseonu DN, Asakura T, Abraham DJ (2011) Crystallographic analysis of human hemoglobin elucidates the structural basis of the potent and dual antisickling activity of pyridyl derivatives of vanillin. Acta Crystallogr D Biol Crystallogr 67(11):920–928
Article
CAS
PubMed
PubMed Central
Google Scholar
Abdulmalik O, Safo MK, Chen Q, Yang J, Brugnara C, Ohene-Frempong K, Abraham DJ, Asakura T (2005) 5-Hydroxymethyl-2-furfural modifies intracellular sickle haemoglobin and inhibits sickling of red blood cells. Br J Haematol 128(4):552–561
Article
CAS
PubMed
Google Scholar
Abraham DJ, Mehanna AS, Wireko FC, Whitney J, Thomas RP, Orringer EP (1991) Vanillin, a potential agent for the treatment of sickle cell anemia. Blood. 77(6):1334–1341
Article
CAS
PubMed
Google Scholar
Abraham EC, Stallings M, Abraham A, Garbutt GJ (1982) Modification of sickle hemoglobin by acetaldehyde and its effect on oxygenation, gelation and sickling. Biochim Biophys Acta 705(1):76–81
Article
CAS
PubMed
Google Scholar
Aruoma OI (1992) Dietary management of sickle cell anaemia with vanillin. Free radical research communications. Free Radic Res Commun 17(5):349–350
Article
CAS
PubMed
Google Scholar
Batchvarova M, Shan S, Zennadi R, Lindgren M, Leitgeb A, Tamsen PS (2013) Sevuparin reduces adhesion of both sickle red cells and leukocytes to endothelial cells in vitro and inhibits vaso-occlusion in vivo. Blood. 122(21):182–182
Article
Google Scholar
Beddell CR, Goodford PJ, Kneen G, White RD, Wilkinson S, Wootton R (1984) Substituted benzaldehydes designed to increase the oxygen affinity of human haemoglobin and inhibit the sickling of sickle erythrocytes. Br J Pharmacol 82(2):397–407
Article
CAS
PubMed
PubMed Central
Google Scholar
Beddell CR, Kneen G, White RD (1979) The anti-sickling activity of a series of aromatic aldehydes [proceedings]. Br J Pharmacol 66(1):70
Google Scholar
Benesch RE, Kwong S, Hudson BB, Krumdieck CL (1988) p-Aminobenzoylpolyglutamates with hydrophobic end groups. A new class of inhibitors of hemoglobin S polymerization. J Biol Chem 263(1):69–71
CAS
PubMed
Google Scholar
Brugnara C, de Franceschi L, Alper SL (1993) Inhibition of Ca2+-dependent K+ transport in sickle cell erythrocytes by clotrimazole and other imidazole derivatives. J Clin Invest 92:520–526
Article
CAS
PubMed
PubMed Central
Google Scholar
Cerami A, Manning JM (1971) Potassium cyanate as an inhibitor of the sickling of erythrocytes in vitro. Proc Natl Acad Sci 68(6):1180–1183
Article
CAS
PubMed
PubMed Central
Google Scholar
Chang H, Ewert SM, Bookchin RM, Nagel RL (1983) Comparative evaluation of fifteen anti-sickling agents. Blood. 61(4):693–704
Article
CAS
PubMed
Google Scholar
Chang J, Patton JT, Sarkar A, Ernst B, Magnani JL, Frenette PS (2010) GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood. 116(10):1779–1786
Article
PubMed
PubMed Central
Google Scholar
Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR, Investigators of the multicenter study of hydroxyurea in sickle cell anemia (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 332(20):1317–1322
Article
CAS
PubMed
Google Scholar
Cheung AT, Chan MS, Ramanujam S, Rangaswami A, Curl K, Franklin P, Wun T (2004) Effects of poloxamer 188 treatment on sickle cell vaso-occlusive crisis: computer-assisted intravital microscopy study. J Investig Med 52(6):402–406
Article
CAS
PubMed
Google Scholar
dos Santos JL, Chin CM (2011) Recent insights on the medicinal chemistry of sickle cell disease. Curr Med Chem 18(15):2339–2358
Article
PubMed
Google Scholar
Dykes G, Crepeau RH, Edelstein SJ (1978) Three-dimensional reconstruction of the fibres of sickle cell haemoglobin. Nature. 272(5653):506–510
Article
CAS
PubMed
Google Scholar
Dykes GW, Crepeau RH, Edelstein SJ (1979) Three-dimensional reconstruction of the 14-filament fibers of hemoglobin S. J Mol Biol 130(4):451–472
Article
CAS
PubMed
Google Scholar
Farthing D, Sica D, Abernathy C, Fakhry I, Roberts JD, Abraham DJ, Swerdlow P (1999) High-performance liquid chromatographic method for determination of vanillin and vanillic acid in human plasma, red blood cells and urine. J Chromatogr B Biomed Sci Appl 726(1–2):303–307
Article
CAS
PubMed
Google Scholar
Fatope MO, Abraham DJ (1987) Design, synthesis, and testing of potential antisickling agents. 10. 2, 2-Dimethylchroman-6-ylalkanoic acids. J Med Chem 30(11):1973–1977
Article
CAS
PubMed
Google Scholar
Ferreira de Melo TR, Chin CM, dos Santos JL (2014) What are the most promising emerging therapies for sickle cell disease? Future Med Chem 6(9):979–982
Article
CAS
PubMed
Google Scholar
Field JJ, Lin G, Okam MM, Majerus E, Keefer J, Onyekwere O, Ross A, Campigotto F, Neuberg D, Linden J, Nathan DG (2013) Sickle cell vaso-occlusion causes activation of iNKT cells that is decreased by the adenosine A2A receptor agonist regadenoson. Blood. 121(17):3329–3334
Article
CAS
PubMed
PubMed Central
Google Scholar
Fung LW, Ho C, Roth EF, Nagel RL (1975) The alkylation of hemoglobin S by nitrogen mustard. High resolution proton nuclear magnetic resonance studies. J Biol Chem 250(12):4786–4789
CAS
PubMed
Google Scholar
Gardos G (1958) The function of calcium in the potassium permeability of human erythrocytes. Biochim Biophys Acta 30(3):653–654
Article
CAS
PubMed
Google Scholar
Gorecki M, Rich A (1977) Sickle hemoglobin aggregation: a new class of inhibitors. Science 196(4295):1216–1219
Article
PubMed
Google Scholar
Grygorczyk R, Schwarz W, Passow H (1984) Ca2+−activated K+ channels in human red cells. Comparison of single-channel currents with ion fluxes. Biophys J 45(4):693–698
Article
CAS
PubMed
PubMed Central
Google Scholar
Hahn EV, Gillespie EB (1927) Sickle cell anemia: report of a case greatly improved by splenectomy. Experimental study of sickle cell formation. Arch Intern Med 39(2):233–254
Article
Google Scholar
Hannemann A, Cytlak UMC, Gbotosho OT, Rees DC, Tewari S, Gibson JS (2014) Effects of o-vanillin on K+ transport of red blood cells from patients with sickle cell disease. Blood Cell Mol Dis 53(1–2):21–26
Article
CAS
Google Scholar
Harris JW (1950) Studies on the destruction of red blood cells. VIII. Molecular orientation in sickle cell hemoglobin solutions. Proc Soc Exp Biol Med 75(1):197–201
Article
CAS
PubMed
Google Scholar
Herrick JB (1910) Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Arch Intern Med 6:517–521
Article
Google Scholar
Hsiao CH, Li W, Lou TF, Baliga BS, Pace BS (2006) Fetal hemoglobin induction by histone deacetylase inhibitors involves generation of reactive oxygen species. Exp Hematol 34(3):264–273
Article
CAS
PubMed
Google Scholar
Ilesanmi OO (2010) Pathological basis of symptoms and crises in sickle cell disorder: implications for counseling and psychotherapy. Hematol Rep 2(1):e2
Article
PubMed
PubMed Central
CAS
Google Scholar
Ingram VM (1956) A specific chemical difference between the globins of normal human and sickle-cell anaemia haemoglobin. Nature. 178(4537):792–794
Article
CAS
PubMed
Google Scholar
Ingram VM (1957) Gene mutations in human haemoglobin: the chemical difference between normal and sickle cell haemoglobin. Nature. 180(4581):326–328
Article
CAS
PubMed
Google Scholar
Ishii TM, Silvia C, Hirschberg B, Bond CT, Adelman JP, Maylie J (1997) A human intermediate conductance calcium-activated potassium channel. Proc Natl Acad Sci 94(21):11651–11656
Article
CAS
PubMed
PubMed Central
Google Scholar
Jenner PM, Hagan EC, Taylor JM, Cook EL, Fitzhugh OG (1964) Food flavourings and compounds of related structure I. Acute oral toxicity. Food Cosmet Toxicol 2:327–343
Article
CAS
Google Scholar
Johnson RM, Feo CJ, Nossal M, Dobo I (1985) Evaluation of covalent antisickling compounds by PO2 scan ektacytometry. Blood. 66(2):432–438
Article
CAS
PubMed
Google Scholar
Kennedy PE, Williams FL, Abraham DJ (1984) Design, synthesis, and testing of potential antisickling agents. 3. Ethacrynic acid. J Med Chem 27(2):103–105
Article
CAS
PubMed
Google Scholar
Kubota S, Yang JT (1977) Oligopeptides as potential antiaggregation agents for deoxyhemoglobin S. Proc Natl Acad Sci 74(12):5431–5434
Article
CAS
PubMed
PubMed Central
Google Scholar
Magbubah Essack M, Bajic V, Radovanovic A (1989) Treatment of sickle cell disease. U.S. Patent 9655905B2
Google Scholar
Mandarino D, Kawar Z, Alvarez R, Falconer D, Rollins SA, Rother RP (2013) Placebo-controlled, double-blind, first-in-human, ascending single dose and multiple dose, healthy subject study of intravenous-administered SelG1, a humanized anti-P-selectin antibody in development for sickle cell disease. Blood 122(21):970–970
Article
Google Scholar
Marotta CA, Wilson JT, Forget BG, Weissman SM (1977) Human beta-globin messenger RNA. III. Nucleotide sequences derived from complementary DNA. J Biol Chem 252(14):5040–5053
CAS
PubMed
Google Scholar
May A, Bellingham AJ, Huehns ER, Beaven GH (1972) Effect of cyanate on sickling. Lancet 299(7752):658–661
Article
Google Scholar
Metcalf B, Chuang C, Dufu K, Patel MP, Silva-Garcia A, Johnson C, Lu Q, Partridge JR, Patskovska L, Patskovsky Y, Almo SC (2017) Discovery of GBT440, an orally bioavailable R-state stabilizer of sickle cell hemoglobin. ACS Med Chem Lett 8(3):321–326
Article
CAS
PubMed
PubMed Central
Google Scholar
Molokie R, Lavelle D, Gowhari M, Pacini M, Krauz L, Hassan J, Ibanez V, Ruiz MA, Ng KP, Woost P, Radivoyevitch T (2017) Oral tetrahydrouridine and decitabine for non-cytotoxic epigenetic gene regulation in sickle cell disease: a randomized phase 1 study. PLoS Med 14(9):e1002382
Article
PubMed
PubMed Central
CAS
Google Scholar
Nakagawa A, Ferrari M, Schleifer G, Cooper MK, Liu C, Yu B, Berra L, Klings ES, Safo RS, Chen Q, Musayev FN (2018) A triazole disulfide compound increases the affinity of hemoglobin for oxygen and reduces the sickling of human sickle cells. Mol Pharm 15(5):1954–1963
Article
CAS
PubMed
PubMed Central
Google Scholar
Nakagawa A, Lui FE, Wassaf D, Yefidoff-Freedman R, Casalena D, Palmer MA, Meadows J, Mozzarelli A, Ronda L, Abdulmalik O, Bloch KD (2014) Identification of a small molecule that increases hemoglobin oxygen affinity and reduces SS erythrocyte sickling. ACS Chem Biol 9(10):2318–2325
Article
CAS
PubMed
PubMed Central
Google Scholar
Nigen AM, Manning JM (1977) Inhibition of erythrocyte sickling in vitro by DL-glyceraldehyde. Proc Natl Acad Sci 74(1):367–371
Article
CAS
PubMed
PubMed Central
Google Scholar
Nigen AM, Njikam N, Lee CK, Manning JM (1974) Studies on the mechanism of action of cyanate in sickle cell disease oxygen affinity and gelling properties of hemoglobin S carbamylated on specific chains. J Biol Chem 249(20):6611–6616
CAS
PubMed
Google Scholar
Nnamani IN, Joshi GS, Danso-Danquah R, Abdulmalik O, Asakura T, Abraham DJ, Safo MK (2008) Pyridyl derivatives of benzaldehyde as potential antisickling agents. Chem Biodivers 5(9):1762–1769
Article
CAS
PubMed
Google Scholar
Oder E, Safo MK, Abdulmalik O, Kato GJ (2016) New developments in anti-sickling agents: can drugs directly prevent the polymerization of sickle haemoglobin in vivo? Br J Haematol 175(1):24–30
Article
CAS
PubMed
PubMed Central
Google Scholar
Oksenberg D, Dufu K, Patel MP, Chuang C, Li Z, Xu Q, Silva-Garcia A, Zhou C, Hutchaleelaha A, Patskovska L, Patskovsky Y (2016) GBT 440 increases haemoglobin oxygen affinity, reduces sickling and prolongs RBC half-life in a murine model of sickle cell disease. Br J Haematol 175(1):141–153
Article
CAS
PubMed
Google Scholar
Omar A, Mahran M, Ghatge M, Bamane F, Ahmed M, El-Araby M, Abdulmalik O, Safo M (2016) Aryloxyalkanoic acids as non-covalent modifiers of the allosteric properties of hemoglobin. Molecules. 21(8):1057
Article
PubMed Central
CAS
Google Scholar
Omar AM, Mahran MA, Ghatge MS, Chowdhury N, Bamane FHA, El-Araby ME, Abdulmalik O, Safo MK (2015) Identification of a novel class of covalent modifiers of hemoglobin as potential antisickling agents. Org Biomol Chem 13(22):6353–6370
Article
CAS
PubMed
PubMed Central
Google Scholar
Pauling L, Itano HA, Singer SJ, Wells IC (1949) Sickle cell anemia, a molecular disease. Science. 110(2865):543–548
Article
CAS
PubMed
Google Scholar
Perutz MF, Fermi G, Abraham DJ, Poyart C, Bursaux E (1986) Hemoglobin as a receptor of drugs and peptides: X-ray studies of the stereochemistry of binding. J Am Chem Soc 108(5):1064–1078
Article
CAS
Google Scholar
Rees DC, Williams TN, Gladwin MT (2010) Sickle-cell disease. Lancet 376(9757):2018–2031
Article
CAS
PubMed
Google Scholar
Roth EF, Nagel RL, Bookchin RM, Grayzel AJ (1972) Nitrogen mustard: an “in vitro” inhibitor of erythrocyte sickling. Biochem Biophys Res Commun 48:612–618
Article
CAS
PubMed
Google Scholar
Russu IM, Lin AK, Yang CP, Ho C (1986) Molecular basis for the antisickling activity of aromatic amino acids and related compounds: a proton nuclear magnetic resonance investigation. Biochemistry. 25(4):808–815
Article
CAS
PubMed
Google Scholar
Safo MK, Abdulmalik O, Danso-Danquah R, Burnett JC, Nokuri S, Joshi GS, Musayev FN, Asakura T, Abraham DJ (2004) Structural basis for the potent antisickling effect of a novel class of five-membered heterocyclic aldehydic compounds. J Med Chem 47(19):4665–4676
Article
CAS
PubMed
Google Scholar
Safo, M.K., Danso-Danquah, R., Nokuri, S., Musayev, F.N., Joshi, G.S., Burnett, J.C. and Abraham, D.J., Xechem International Inc, 2007. Anti-sickling agents. US Patent 7,160,910
Google Scholar
Safo MK, Kato GJ (2014) Therapeutic strategies to alter the oxygen affinity of sickle hemoglobin. Hematology/Oncology Clinics. 28(2):217–231
Article
Google Scholar
Saunthararajah Y, Lavelle D, DeSimone J (2004) DNA hypo-methylating agents and sickle cell disease. Br J Haematol 126(5):629–636
Article
CAS
PubMed
Google Scholar
Strand LP, Scheline RR (1975) The metabolism of vanillin and isovanillin in the rat. Xenobiotica. 5(1):49–63
Article
CAS
PubMed
Google Scholar
Taliaferro WH, Huck JG (1923) The inheritance of sickle-cell anaemia in man. Genetics 8(6):594–598
CAS
PubMed
PubMed Central
Google Scholar
Telen MJ (2016) Beyond hydroxyurea: new and old drugs in the pipeline for sickle cell disease. Blood. 127(7):810–819
Article
CAS
PubMed
PubMed Central
Google Scholar
Walder JA, Walder RY, Arnone A (1980) Development of antisickling compounds that chemically modify hemoglobin S specifically within the 2, 3-diphosphoglycerate binding site. J Mol Biol 141(2):195–216
Article
CAS
PubMed
Google Scholar
Walder JA, Zaugg RH, Walder RY, Steele JM, Klotz IM (1979) Diaspirins that crosslink. Beta. Chains of hemoglobin: bis (3, 5-dibromosalicyl) succinate and bis (3, 5-dibromosalicyl) fumarate. Biochemistry. 18(20):4265–4270
Article
CAS
PubMed
Google Scholar
Wishner BC, Ward KB, Lattman EE, Love WE (1975) Crystal structure of sickle-cell deoxyhemoglobin at 5 Å resolution. J Mol Biol 98(1):179–194
Article
CAS
PubMed
Google Scholar
Zaugg RH, Walder JT, Klotz IM (1977) Schiff base adducts of hemoglobin. Modifications that inhibit erythrocyte sickling. J Biol Chem 252(23):8542–8548
CAS
PubMed
Google Scholar
Zhang C, Li X, Lian L, Chen Q, Abdulmalik O, Vassilev V, Lai CS, Asakura T (2004) Anti-sickling effect of MX-1520, a prodrug of vanillin: an in vivo study using rodents. Br J Haematol 125(6):788–795
Article
CAS
PubMed
Google Scholar