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|Title:||The rare α-Thalassemia-1 of Blacks is a ζα-Thalassemia-1 associated with a deletion of all α- and ζ-Globin genes|
|Authors:||Felice, Alex E.|
Cleek, M. P.
McKie, Kathleen Mood
McKie, Virgil C.
Huisman, Titus Hendrik Jan
Thalassemia in children
|Publisher:||The American Society of Hematology|
|Citation:||Felice, A. E., Cleek, M. P., McKie, K., McKie, V., & Huisman, T. H. J. (1984). The rare α-Thalassemia-1 of Blacks is a ζα-Thalassemia-1 associated with a deletion of all α- and ζ-Globin genes. Blood, 63(5), 1253-1257.|
|Abstract:||Restriction endonuclease mapping with α and ζ-globin gene probes showed differences between the α-thalassemia-1 (α-thal-1) condition in 2 patients with HbH disease. One patient had the rare black type of α-thal-1 together with α-thal-2 and HbS heterozygosities. The second patient was a Laotian child with HbE, Hb Constant Spring (α-thal-2), and α-thal-1 heterozygosities. The diagnoses were based on clinical, hematologic, and biochemical data. Whereas DNA fragments hybridizing to a ζ-probe were obtained from the Laotian type of α-thal-1, neither α- nor ζ-gene fragments could be identified deriving from the black type of α-thal-1. Therefore, the black type of α-thal-1 is associated with a deletion of the entire ζ2-ψζ-ψα-α2-α1 gene complex and can be considered a ζα-thal-1. It is likely that homozygosity for such a condition will lead to embryonic wastage, explaining the absence of hydrops fetalis in blacks.|
|Appears in Collections:||Scholarly Works - FacM&SPB|
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