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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
1997-5-7
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pubmed:abstractText |
Five factors have been hypothesized to influence the 20-fold variation in fetal haemoglobin (Hb F) levels in sickle cell anaemia (SS): age sex, alpha-globin gene number, beta-globin haplotype, and the X-linked F-cell production locus (FCP) that regulates the production of Hb F containing erythrocytes (F cells). We analysed the association of these factors with Hb F levels in 112 SS patients living in France who are homozygous for the three common African beta-globin haplotypes (Benin, Bantu or Central African Republic and Senegal). We found that: (1) FCP accounts for about 40% of the overall variation in Hb F levels, (2) when the FCP influence is removed, beta-globin haplotype is associated with 14% of the remaining Hb F variation, and (3) the other factors have little influence. Comparison with our previous study of SS individuals in Jamaica leads to the following conclusions: (1) the X-linked FCP locus is a major determinant of Hb F levels in SS disease, (2) factors linked to the beta-globin haplotype have only a small effect on the variation in Hb F levels, in either the homozygous or heterozygous state, and (3)approximately half of the variation in Hb F levels still remains to be explained.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
0007-1048
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
96
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
806-14
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pubmed:dateRevised |
2010-11-18
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pubmed:meshHeading |
pubmed-meshheading:9074425-Adolescent,
pubmed-meshheading:9074425-Adult,
pubmed-meshheading:9074425-Anemia, Sickle Cell,
pubmed-meshheading:9074425-Child,
pubmed-meshheading:9074425-Child, Preschool,
pubmed-meshheading:9074425-Female,
pubmed-meshheading:9074425-Fetal Hemoglobin,
pubmed-meshheading:9074425-Genetic Linkage,
pubmed-meshheading:9074425-Globins,
pubmed-meshheading:9074425-Haplotypes,
pubmed-meshheading:9074425-Humans,
pubmed-meshheading:9074425-Infant,
pubmed-meshheading:9074425-Male,
pubmed-meshheading:9074425-Phenotype,
pubmed-meshheading:9074425-Regression Analysis,
pubmed-meshheading:9074425-Reticulocytes,
pubmed-meshheading:9074425-X Chromosome
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pubmed:year |
1997
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pubmed:articleTitle |
The relative importance of the X-linked FCP locus and beta-globin haplotypes in determining haemoglobin F levels: a study of SS patients homozygous for beta S haplotypes.
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pubmed:affiliation |
Department of Pediatrics, Johns Hopkins University, Baltimore, Maryland, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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