Functional behaviour of dilute solutions of Hb-Kempsey: beta 99 (G-1) Asp--Asn
Rabino Massa, E.; Ricco, G.; Polimeni, C.; Reddavid, M.
Bollettino della Societa Italiana di Biologia Sperimentale 68(3): 167-174
1992
ISSN/ISBN: 0037-8771 PMID: 1389072 Document Number: 389583
The authors report that a diluted solution of Hb-Kempsey, beta 99 (G-1) Asp-Asn, can be chromatographically separated from the coexistent Hb-A and functionally examined if progressively depleted in O-2 by bubbling pure nitrogen in the solution. Next, at fixed times, the O-2 saturations of Hb are compared with the pO-2s measured. Hb-Kempsey has a p50 of 1 torr, with an n-value of 1 and a Bohr effect of -0.2. Normal Hb-A of the same patient, examined with identical methods, presents: p50 = 4.5 torr; n = 2.7; Bohr effect = -0.412. Therefore, Hb-Kempsey is strongly hyperaffine, does not display any heme-heme interaction, and has a half-normal Bohr effect.