PMID: 4123929Apr 1, 1973Paper

Structure of fibers of sickle cell hemoglobin

Proceedings of the National Academy of Sciences of the United States of America
S J EdelsteinR H Crepeau

Abstract

Electron microscope studies have been conducted on individual fibers of human deoxyhemoglobin S (sickle cell hemoglobin). The fibers are obtained by injection of gelled samples into a large excess of glutaraldehyde, which quickly stabilizes the fibers by cross-linking. The fibers are negatively stained with phosphotungstic acid or shadowed with platinum-carbon. The fibers are approximately 200 A in diameter, and display long and short helical striations with an opposite handedness. The long striations occur at an angle of about 15 degrees from the fiber axis, and complete one turn around the helix at a distance of about 2 x 10(3) A along the fiber axis. The short striations occur at an angle of about 80 degrees from the fiber axis, with a spacing of about 65 A, and complete one turn around the helix at a distance along the fiber axis of about 130 A. The structure of the fiber appears to be a sextuple helix in terms of the long striations, and a double helix in terms of the short striations. The shadowed samples are consistent with a left-handed screw sense for the short striations, thus implying a right-handed sense for the long striations. A structural model incorporating these features is compatible with the atomic structure ...Continue Reading

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Citations

Feb 26, 1979·Biochimica Et Biophysica Acta·T E HoustonT H Huisman
Nov 15, 1979·Journal of Molecular Biology·S J Edelstein, R H Crepeau
Jan 1, 1982·Comparative Biochemistry and Physiology. A, Comparative Physiology·C F SimpsonE R Jacobson
Jun 1, 1997·Blood Reviews·S C Davies, M Roberts-Harewood
Dec 1, 1973·Proceedings of the National Academy of Sciences of the United States of America·J HofrichterW A Eaton
Dec 1, 1974·Proceedings of the National Academy of Sciences of the United States of America·D ElbaumT T Herskovits
Apr 1, 1974·Proceedings of the National Academy of Sciences of the United States of America·W W WilsonC S Johnson
Feb 1, 1991·The Journal of Cell Biology·F M ButterworthV S Burde
Nov 29, 1974·Annals of the New York Academy of Sciences·W J Taylor, C W Easley
Nov 29, 1974·Annals of the New York Academy of Sciences·C F Simpson, W J Taylor
Jul 5, 1979·Journal of Molecular Biology·R L GirlingT H Huisman
Jun 13, 1979·Biochemical and Biophysical Research Communications·R L GirlingT H Huisman
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