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Citations to this article

Functional and physicochemical studies of hemoglobin St. Louis beta 28 (B10) Leu replaced by Gln: a variant with ferric beta heme iron.
J Thillet, … , M Seligmann, J Rosa
J Thillet, … , M Seligmann, J Rosa
Published November 1, 1976
Citation Information: J Clin Invest. 1976;58(5):1098-1106. https://doi.org/10.1172/JCI108561.
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Research Article

Functional and physicochemical studies of hemoglobin St. Louis beta 28 (B10) Leu replaced by Gln: a variant with ferric beta heme iron.

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Abstract

Studies have been performed on a 20-yr-old man exhibiting methemoglobinemia and a severe hemolytic anemia involving formation of Heinz bodies. This condition was due to an abnormal Hb present in the red cells of the proband: Hb St. Louis, beta 28 (B10) replaced by Gln, whose structural characteristics have been previously reported. This unstable Hb represented 30% of the total and was isolated by starch block electrophoresis at pH 8.6. Electrophoretic and spectral studies showed Hb St. Louis to be a valency hybird, alpha 2 beta 2+. The presence of hemichrome in this Hb was detected by electron paramagnetic resonance studies. During this study, an electrophoretic technique was developed that allows study of the mobility of hemichrome. Oxygen equilibria performed on purified Hb St. Louis revealed a high oxygen affinity and a markedly reduced cooperativity. The Bohr effect was normal, but the interaction of this hemoglobin with 2,3-diphosphoglycerate was decreased. The oxidation rate of Hb St. Louis was normal. Hb St. Louis was completely reduced by dithionite and ferrous citrate, and the functional properties of this reduced form were normal. In contrast, Hb St. Louis was only partially reduced by diaphorase. The mechanism of the oxidation of Hb St. Louis therefore appears to differ markedly from that postulated for other Hbs M.

Authors

J Thillet, M Cohen-Solal, M Seligmann, J Rosa

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Total citations by year

Year: 2016 2013 1999 1997 1993 1992 1986 1984 1983 1980 1978 1976 Total
Citations: 1 1 2 1 1 1 1 1 1 1 1 1 13
Citation information
This citation data is accumulated from CrossRef, which receives citation information from participating publishers, including this journal. Not all publishers participate in CrossRef, so this information is not comprehensive. Additionally, data may not reflect the most current citations to this article, and the data may differ from citation information available from other sources (for example, Google Scholar, Web of Science, and Scopus).

Citations to this article in year 2013 (1)

Title and authors Publication Year
Transient Neonatal Cyanosis Associated With a New Hb F Variant: Hb F Viseu
C Bento, TM Maia, I Carvalhais, F Moita, G Abreu, L Relvas, A Pereira, JF Neves, ML Ribeiro
Journal of Pediatric Hematology/Oncology 2013

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