Document Detail


Kinetic analysis in single, intact cells by microspectrophotometry: evidence for two populations of erythrocytes in an individual heterozygous for glucose-6-phosphate dehydrogenase deficiency.
MedLine Citation:
PMID:  3788959     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A microspectrophotometric technique was used to measure the kinetics of methemoglobin reduction in intact, unaltered human erythrocytes. Reduction was catalyzed by endogenous NADPH-methemoglobin reductase in the presence of Nile Blue. The technique was applied to the study of erythrocytes from a female donor with decreased glucose-6-phosphate dehydrogenase (G-6-PD) activity. The individual was shown to be heterozygous for deficiency of G-6-PD. The kinetic study revealed two distinct populations of erythrocytes that were nearly equal in number. One cell population showed reduction rates between 0 and 25% of normal, whereas the second cell population displayed rates within the range seen for normal cells. Single-cell indices of cell size, cell hemoglobin content, and ratio of cell hemoglobin to cell size did not correlate with single-cell reduction rates and were not significantly different between the two populations. These results provide quantitative support for the X-inactivation hypothesis in G-6-PD deficiency.
Authors:
R A Ashmun; D E Hultquist; J S Schultz
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  American journal of hematology     Volume:  23     ISSN:  0361-8609     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  1986 Dec 
Date Detail:
Created Date:  1986-12-22     Completed Date:  1986-12-22     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  311-6     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Erythrocytes / classification*,  metabolism
Female
Glucosephosphate Dehydrogenase / blood
Glucosephosphate Dehydrogenase Deficiency / blood,  genetics*
Heterozygote
Humans
Kinetics
Male
Methemoglobin / metabolism
NADP / metabolism
Oxidation-Reduction
Spectrophotometry
Grant Support
ID/Acronym/Agency:
2S07-RR077050/RR/NCRR NIH HHS; AM-09250/AM/NIADDK NIH HHS; AM-26858/AM/NIADDK NIH HHS
Chemical
Reg. No./Substance:
53-59-8/NADP; 9008-37-1/Methemoglobin; EC 1.1.1.49/Glucosephosphate Dehydrogenase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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