Document Detail


X-linked dominant control of F-cells in normal adult life: characterization of the Swiss type as hereditary persistence of fetal hemoglobin regulated dominantly by gene(s) on X chromosome.
MedLine Citation:
PMID:  2461753     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fetal hemoglobin (HbF) levels determined in healthy Japanese adults ranged from 0.3% to 16.0% as F cells and 0.17% to 2.28% as HbF content, which were the same as those obtained in other countries. The frequency distribution of 300 healthy adults with various numbers of F cells consisted statistically of two different groups, low and high F-cell groups. Individuals with greater than or equal to 4.4% of F cells (HbF about 0.7%) were defined as the high F-cell trait, which accounted for 11.3% of males and 20.7% of females. Family studies of 21 probands with this trait and sex-different frequency analyses in the population and probands revealed X-linked dominant inheritance. Two other families of the trait associated with color blindness were described, although no definitive evidence for linkage was obtained between the two. A review of population and family studies reported in the literature indicated that persons with Swiss-type hereditary persistence of fetal hemoglobin (HPFH) are of the same kind as this trait in their incidence and inheritance form, but represent a portion of the trait with higher levels of HbF or F cells. The existence of X chromosome-localized regulatory gene(s) for the developmental switch of human Hb production is discussed.
Authors:
K Miyoshi; Y Kaneto; H Kawai; H Ohchi; S Niki; K Hasegawa; A Shirakami; T Yamano
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Blood     Volume:  72     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  1988 Dec 
Date Detail:
Created Date:  1989-01-18     Completed Date:  1989-01-18     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1854-60     Citation Subset:  AIM; IM    
Affiliation:
First Department of Internal Medicine, School of Medicine, University of Tokushima, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adult
Color Vision Defects / genetics
Erythrocytes / analysis
Female
Fetal Hemoglobin / analysis*
Genes, Dominant
Hemoglobinopathies / epidemiology,  genetics*
Humans
Japan
Linkage (Genetics)
Male
Pedigree
X Chromosome*
Chemical
Reg. No./Substance:
9034-63-3/Fetal Hemoglobin

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


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