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.

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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Year: 2016 2013 1999 1997 1993 1992 1986 1984 1983 1980 1978 1976 Total
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Citations to this article (13)

Title and authors Publication Year
Five Rare β Globin Chain Hemoglobin Variants in India
RB Colah, A Nadkarni, A Gorakshakar, P Sawant, M Gorivale, P Mehta, M Sawant, K Ghosh 		Indian Journal of Hematology and Blood Transfusion 2016
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
IS IT DOMINANTLY INHERITED beta THALASSAEMIA OR JUST A beta-CHAIN VARIANT THAT IS HIGHLY UNSTABLE?
SL Thein 		British Journal of Haematology 1999
Hb Chile [β28(B10)Leu→Met]: An unstable hemoglobin associated with chronic methemoglobinemia and sulfonamide or methylene blue-induced hemolytic anemia
R Hojas-Bernal, P McNab-Martin, VF Fairbanks, MW Holmes, JD Hoyer, DJ McCormick, KS Kubik 		Hemoglobin 1999
International Hemoglobin Information Center Variant List
MF Carver, TH Huisman 		Hemoglobin 1997
HB Higashitochigi (HB HT) [β24(B6) or β25(B7) Glycine Deleted]: A New Unstable Variant Expressing Cyanosis
K Fujisawa, Y Yamashiro, Y Hattori, Y Ohba, T Kajita, S Kageyama, J Arita 		Hemoglobin 1993
DOMINANT β THALASSAEMIA: MOLECULAR BASIS AND PATHOPHYSIOLOGY
SL Thein 		British Journal of Haematology 1992
Hb Saint Louis or α 2 β 2 28(B10)Leu→Gln in a Czechoslovakian Male
BF Wiedermann, K Indrak, JB Wilson, BB Webber, KG Yang, F Kutlar, A Kutlar, TH Huisman 		Hemoglobin 1986
Structural study of hemoglobin Hazebrouck, β38(C4)Thr → Pro
Y Blouquit, J Delanoe-Garin, C Lacombe, N Arous, Y Cayre, J Peduzzi, F Braconnier, F Galacteros 		FEBS Letters 1984
Thalassemic hemoglobinopathies
MH Steinberg, JG Adams 		The American Journal of Pathology 1983
Oxidation properties of two hemoglobin variants with their mutation localized in the heme pocket: Hb castilla β32 (B14) and Hb toulouse β66 (E10) , and abnormal functional properties of Hb castilla
J Thillet, MC Garel, R Bierme, J Rosa 		Biochimica et Biophysica Acta (BBA) - Protein Structure 1980
Carbon-13 NMR studies of C2H5N13C bound to hemoproteins — Evidence for a different distal proteic environment in β-chains either isolated or within human hemoglobin
D Mansuy, J Thillet, B Cendrier, JY Lallemand, JC Chottard 		Biochemical and Biophysical Research Communications 1978
Hemoglobin St. Louis [beta 28 (B10) Leu replaced by Gln]: crystal structure of the fully reduced (deoxy) form
NL Anderson 		Journal of Clinical Investigation 1976

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