Fetal hemoglobin levels and beta s globin haplotypes in an Indian population with sickle cell disease
Blood
; 69(6): 1742-6, June 1987.
Article
em En
| MedCarib
| ID: med-15878
Biblioteca responsável:
JM3.1
Localização: JM3.1; RB145.A1B5
ABSTRACT
To further explore the cause for variation in hemoglobin F (HbF) levels in sickle cell disease, the á globin restriction-fragment length polymorphism haplotypes were determined in a total of 303 (126SS, 141AS, 17Sá§, 7Aá§, and 12AA) Indians from the state of Orissa. The ás globin gene was found to be linked almost exclusively to a ás haplotype (+++-++-), which is also common in Saudi Arabian patients from the Eastern province (referred to as the Asian ás haplotype). By contrast, the majority of áA and ᧠thalassemia globin genes are linked to hoplotypes common in all European and Asian populations (+-----[+/-];--++-++). Family studies showed that there is a genetic factor elevating HbF levels dominantly in homozygotes (SS). This factor appears to be related to the Asian ás globin haplotype, and a mechanism for its action is discussed. There is also a high prevalence of an independent Swiss type hereditary presistance of fetal hemoglobin (HPFH) determinant active in both the sickle cell trait and in sickle cell disease.(AU)
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Coleções:
01-internacional
Base de dados:
MedCarib
Assunto principal:
Hemoglobina Fetal
/
Globinas
/
Anemia Falciforme
Tipo de estudo:
Risk_factors_studies
Limite:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Humans
País/Região como assunto:
Asia
/
Caribe ingles
/
Jamaica
Idioma:
En
Revista:
Blood
/
Blood (Online)
Ano de publicação:
1987
Tipo de documento:
Article