Hb Hope, beta 136(H14)Gly----Asp, in a diabetic Japanese female and its functional characterization.
A beta-variant hemoglobin, first misjudged as a marked elevation of Hb A1, was found in a 68-year-old Japanese female with diabetes mellitus. This hemoglobin was isolated by Bio-Rex 70 chromatography combined with chromatofocusing, and was found to be Hb Hope, beta 136(H14)Gly----Asp, by classical and high performance liquid chromatographic peptide mapping techniques. Intrinsic oxygen affinity of this hemoglobin was approximately one-third as compared with that of Hb A0. This property was still observed in the constituent beta subunits isolated. Effects of such allosteric effectors as H+ (at a fixed concentration of Cl-), anion (Cl-), 2,3-diphosphoglycerate and carbon dioxide were more or less depressed. Among others, a marked reduction in the carbamate effect should be noted in a structural interpretation of the functional modifications. Subunit cooperativity, on the contrary, was not different from that in Hb A0 (n = 2.8-2.9). Explanation of these altered functions were attempted on the basis of the altered structure. The reduced stability of Hb Hope is also described.
Enoki
Y
Y
Second Department of Physiology, Nara Medical University, Japan.
Ohga
Y
Y
Furukawa
K
K
Takaya
A
A
Sakata
S
S
Kohzuki
H
H
Shimizu
S
S
Tsujii
T
T
D000368:Aged; D000596:Amino Acids / Q000032:analysis; D002852:Chromatography, Ion Exchange; D003920:Diabetes Mellitus / Q000097:blood* / Q000235:genetics; D004592:Electrophoresis, Starch Gel; D005260:Female; D006441:Hemoglobin A / Q000302:isolation & purification; D006455:Hemoglobins, Abnormal / Q000302:isolation & purification*; D006579:Heterozygote; D006801:Humans; D007564:Japan; D010100:Oxygen / Q000032:analysis
D000596:Amino Acids; D006455:Hemoglobins, Abnormal; D006441:Hemoglobin A; C038623:hemoglobin Hope; D010100:Oxygen