TY - JOUR
AU - HUEHNS, E. R.
AB - THE MECHANISM AND PREVENTION OF SICKLING A. May A E. R. Huehns The amino acid substitution has very little effect on the oxy form of Hb S (Perutz et al. 1951; Asakura et al. 1973; DeSimone & Adams, 1974; Yip et al. 1974) and at low haemo- globin concentration the deoxygenated Hb S has the same gross structure (Ho et al. 1974; Wishner et al. 1975) and behaviour as Hb A, so there is little intrinsic intramolecular THE MECHANISM AND PREVENTION effect of the substitution. However, when the concentration OF SICKLING of deoxygenated Hb S is sufficiently great its properties differ drastically from those of deoxygenated Hb A because of a A. MAY* Ph.D. polymerization of the molecules into filaments of indeter- minately high molecular weight. These associate laterally E. R. HUEHNS M.D. PhD. to form fibres and bundles of fibres which, in vivo, distort Department of Clinical Haematology the red cell into the characteristic sickle shape. The rigidity University College Hospital Medical School, London and distortion of the sickle cells lead to their premature destruction and to anaemia but, more importantly, they 1 The structure of the polymer increase the viscosity of the deoxygenated blood, impeding a Direct 
TI - THE MECHANISM AND PREVENTION OF SICKLING
JO - British Medical Bulletin
DO - 10.1093/oxfordjournals.bmb.a071367
DA - 1976-09-01
UR - https://www.deepdyve.com/lp/oxford-university-press/the-mechanism-and-prevention-of-sickling-a7RjvsbtEq
SP - 223
EP - 233
VL - 32
IS - 3
DP - DeepDyve
ER -