+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Oxygen equilibrium characteristics of abnormal hemoglobins. Hirose (alpha-2-beta-2-37Ser), L Ferrara (alpha-2-47-Gly-beta-2), Broussais (alpha-2-90-Asn-beta-2), and Dhofar (alpha-2-beta-2-58Arg)

Oxygen equilibrium characteristics of abnormal hemoglobins. Hirose (alpha-2-beta-2-37Ser), L Ferrara (alpha-2-47-Gly-beta-2), Broussais (alpha-2-90-Asn-beta-2), and Dhofar (alpha-2-beta-2-58Arg)

Journal of Clinical Investigation 51(10): 2520-2529

The oxygen equilibrium characteristics of four structural variants of hemoglobin A were correlated with their amino acid substitutions. Hemoglobin Dhofar, in which the proline at E2(58)beta is replaced by arginine, had normal oxygen equilibrium characteristics. Hemoglobin L Ferrara. in which the aspartic acid at CD5(47)alpha is replaced by glycine, and hemoglobin Broussais, in which the lysine at FG2(90)alpha is replaced by asparagine, both showed a slightly elevated oxygen affinity; nevertheless both demonstrated a normal heme-heme interaction and a normal Bohr effect. Hemoglobin Hirose, in which the tryptophan at C3 (37)beta is replaced by serine, showed abnormalities of all oxygen equilibrium characteristics; i.e., increased oxygen affinity, diminished heme-heme interaction, and reduced Bohr effect.These results suggest that aspartic acid at CD5(47)alpha and lysine at FG2(90)alpha are involved in the function of the hemoglobin molecule, despite the fact that these positions are not located directly in the heme or the alpha-beta-contact regions. Tryptophan at C3(37)beta is located at contact between alpha(1)- and beta(2)-subunits. It is suggested that the substitution by serine might disturb the quarternary structure of the mutant hemoglobin molecule during transition from oxy-form to deoxy-form resulting in an alteration of the heme function.

(PDF emailed within 0-6 h: $19.90)

Accession: 043868800

Download citation: RISBibTeXText

PMID: 5056652

DOI: 10.1172/JCI107067

Related references

Pharmacological characterization of recombinant human neuronal nicotinic acetylcholine receptors h alpha 2 beta 2, h alpha 2 beta 4, h alpha 3 beta 2, h alpha 3 beta 4, h alpha 4 beta 2, h alpha 4 beta 4 and h alpha 7 expressed in Xenopus oocytes. Journal of Pharmacology and Experimental Therapeutics 280(1): 346-356, 1997

Engineered topographic determinants with alpha beta, beta alpha beta, and beta alpha beta alpha topologies show high affinity binding to native protein antigen (lactate dehydrogenase-C4). Journal of Biological Chemistry 268(34): 25285-25295, 1993

Conjugate addition reactions of alpha-aminoalkylcuprates with alpha, beta-alkenyl-, alpha,beta-alkynyl-, alpha,beta-beta,gamma-allenyl-, and alpha,beta-gamma,delta-dienyl carboxylic acid derivatives, nitriles, and sulfoxides. Journal of Organic Chemistry 65(25): 8715-8724, 2000

The obligatory intermediacy of 16,17 alpha- and 16,17 beta-epoxides in the biotransformation of androsta-5,16-dien-3 beta-ol to androst-5-ene-3 beta, 16 alpha, 17 beta- and -3 beta, 16 beta, 17 alpha-triols by male rat liver microsomes. Journal of Biological Chemistry 260(15): 8716-8720, 1985

Comparative structure of human neuronal alpha 2-alpha 7 and beta 2-beta 4 nicotinic acetylcholine receptor subunits and functional expression of the alpha 2, alpha 3, alpha 4, alpha 7, beta 2, and beta 4 subunits. Journal of Molecular Neuroscience 7(3): 217-228, 1996

Synthetic studies on the construction of the fundamental skeleton of erythroxydiol part 10 syntheses of 3a 10a di methyl 3a beta 4a alpha 6a beta 10a alpha 10b beta per hydro cyclo propa j phenanthrene 5 8 dione and 8 alpha benzoyloxy 3a 10a dimethyl 3a beta 4a alpha 6a beta 10a alpha 10b beta per hydro cyclo propa j phenanthren 3 one. Journal of the Chemical Society Perkin Transactions I (23): 2581-2584, 1977

Rapid, regiospecific syntheses of deuterium substituted 6-(fluorine-18) fluorodopamine (alpha,alpha-D2',beta,beta-D2 and alpha,alpha,beta,beta-D-4) for mechanistic studies with positron emission tomography. Journal of Labelled Compounds and Radiopharmaceuticals 33(7): 645-654, 1993

Confirmation of the structure of fibraurin as 2r 2 alpha 6a alpha 7 alpha 7a beta 8a beta 9 beta 9a alpha 9b beta 2 furan 3 yl 7 hydroxy 6a 9b dimethyl 1 2 6 6a 7 7a 8a 9 9a 9b decahydro 9 7 epoxymethano 4h oxireno 6 7 naphtho 2 1 c pyran 4 11 dione. Australian Journal of Chemistry 39(1): 177-182, 1986

Polarized expression of integrin receptors (alpha 6 beta 4, alpha 2 beta 1, alpha 3 beta 1, and alpha v beta 5) and their relationship with the cytoskeleton and basement membrane matrix in cultured human keratinocytes. Journal of Cell Biology 112(4): 761-773, 1991

Expression of laminin and alpha-2-beta-1, alpha-3-beta-1, alpha-6-beta-1 and alpha-6-beta-4 integrins in tumor development of two human colon adenocarcinoma cell clones xenografted in the immunosuppressed newborn rat and displaying low and high metastatic abilities respectively. Clinical & Experimental Metastasis 12(5): 40, 1994

Syntheses of (14 beta,15 beta,16 beta,17 alpha)- and (14 beta,15 alpha,16 alpha,17 alpha)-1,3,5(10)-estratriene-2,3,14,15,16,17- hexaols, possible candidates for the Inagami--Tamura endogenous digitalis-like factor, and their activity. Bioscience, Biotechnology, and Biochemistry 60(3): 405-410, 1996

Comparison of the chemical reactivities and antineoplastic activities of alpha,beta-, alpha,beta'-, beta,beta'-, and alpha,alpha'-bis[[[(2-propylamino)carbonyl]oxy]methyl]-substituted pyrroles. Journal of Medicinal Chemistry 29(11): 2392-2395, 1986

Distinct structural requirements for interaction of the integrins alpha-5-beta-1, alpha-v-beta-3, alpha-v-beta-5, and alpha-v-beta-6 with the central cell binding domain in fibronectin. Molecular Biology of the Cell 6(SUPPL ): 388A, 1995

C-19-steroid 7 alpha-hydroxylation by rat testes. Isolation and identification of: 7 alpha, 17 beta-dihydroxy-5 alpha-androstan-3-one, 5 alpha-androstan-3 alpha, 7 alpha, 17 beta-triol and 5 alpha-androstane-3 beta, 7 alpha, 17 beta-triol. Journal of Steroid Biochemistry 17(1): 85-88, 1982

Significance of bile alcohol in urine of a patient with cholestasis: Identification of 5.BETA.-cholestane-3.ALPHA.,7.ALPHA.,12.ALPHA.,26,27-pentol (5.BETA.-cyprinol) and 5.BETA.-cholestane-3.ALPHA.,7.ALPHA.,12.ALPHA.,26-tetrol (27-deoxy-5.BETA.-cyprinol). Chemical & Pharmaceutical Bulletin 32(7): 2874-2877, 1984