[Oxygen-binding properties of blood in hemoglobinosis M Boston detected in the USSR for the first time].
Hemoglobin M-Boston comprising 30% of the total blood Hb was detected in two members of a Russian family. The stages of its identification were described. An analysis was made of probands' whole blood oxy-Hb dissociation curves. At pO2 = 100 mm Hg oxygenation of Hb M-Boston beta-chains was 44% only, while mutant alpha-chains were completely oxidated. Despite a significant decrease in arterial blood oxygen content arteriovenous variation by O2 content at pO2 differential from 100 to 40 mm Hg is within the normal level due to various directions of changes in P50 and Hill's coefficient of probands' whole blood. The authors consider that pronounced cyanosis in Hb M-Boston carriers is not the consequence of tissue hypoxia but is caused by a higher content of nonfunctioning Hb M-Boston that changes the blood color.
Dmitrieva
M G
MG
Kazanets
E G
EG
Molchanova
T P
TP
Karpova
I V
IV
Sevast'ianova
M G
MG
Andreeva
A P
AP
D000293:Adolescent; D000328:Adult; D003490:Cyanosis / Q000097:blood* / Q000175:diagnosis / Q000235:genetics; D005260:Female; D006449:Hemoglobin M / Q000032:analysis*; D006453:Hemoglobinopathies / Q000097:blood* / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007623:Kazakhstan; D008297:Male; D010100:Oxygen / Q000097:blood*; D010108:Oxyhemoglobins / Q000378:metabolism*
D010108:Oxyhemoglobins; C027541:hemoglobin M Boston; D006449:Hemoglobin M; D010100:Oxygen
Kislorodsviazyvaiushchie svoĭstva krovi pri gemoglobinoze M-Boston, vpervye vyiavlennom v SSSR.