Biomaterial Database

Decreased reduced glutathione and glutathione reductase activity in subjects with hemoglobin C. 1991

S Saad, and S I Salles, and P E Velho

Department of Clinical Medicine, State University of Campinas, UNICAMP-Brazil.

Erythrocyte catalase, reduced glutathione, glutathione peroxidase and glutathione reductase were determined in 17 normal black controls, 8 subjects with Hb AC, 12 with Hb SC, 1 with Hb CC and 18 patients with sickle cell anemia. Catalase and glutathione peroxidase activities were decreased in sickle cell anemia. Reduced glutathione and glutathione reductase activity were significantly lower in subjects with Hb C (AC, CC, SC). Differences were observed between Hb C, Hb S and Hb A as regards red cell dehydration, intracellular crystallization, enhanced potassium efflux, an increased number of titratable SH groups in Hb C and the binding of Hb C to band 3 on the inner membrane surface. A decrease in reduced glutathione, probably due to inhibition or decreased synthesis of glutathione reductase, was also observed. All these factors may determine oxidation of Hb C, possibly contributing to the hemolysis in patients with Hb C disease.

UI	MeSH Term	Description	Entries
D010084	Oxidation-Reduction	A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).	Redox,Oxidation Reduction
D004912	Erythrocytes	Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.	Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D005978	Glutathione	A tripeptide with many roles in cells. It conjugates to drugs to make them more soluble for excretion, is a cofactor for some enzymes, is involved in protein disulfide bond rearrangement and reduces peroxides.	Reduced Glutathione,gamma-L-Glu-L-Cys-Gly,gamma-L-Glutamyl-L-Cysteinylglycine,Glutathione, Reduced,gamma L Glu L Cys Gly,gamma L Glutamyl L Cysteinylglycine
D005980	Glutathione Reductase	Catalyzes the oxidation of GLUTATHIONE to GLUTATHIONE DISULFIDE in the presence of NADP+. Deficiency in the enzyme is associated with HEMOLYTIC ANEMIA. Formerly listed as EC 1.6.4.2.	Glutathione-Disulfide Reductase,Reductase, Glutathione,Reductase, Glutathione-Disulfide
D006441	Hemoglobin A	Normal adult human hemoglobin. The globin moiety consists of two alpha and two beta chains.
D006444	Hemoglobin C	A commonly occurring abnormal hemoglobin in which lysine replaces a glutamic acid residue at the sixth position of the beta chains. It results in reduced plasticity of erythrocytes.
D006451	Hemoglobin, Sickle	An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia.	Hemoglobin S,Deoxygenated Sickle Hemoglobin,Deoxyhemoglobin S,Hemoglobin SS,Hemoglobin, Deoxygenated Sickle,SS, Hemoglobin,Sickle Hemoglobin,Sickle Hemoglobin, Deoxygenated
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults