Bouhassira E E, Lachman H, Krishnamoorthy R, Labie D, Nagel R L
Division of Hematology, Albert Einstein College of Medicine, Bronx, NY 10461.
J Clin Invest. 1989 Jun;83(6):2070-3. doi: 10.1172/JCI114118.
Cloning and sequencing of the gamma-globin gene of a sickle cell anemia patient homozygous for the Bantu haplotype has revealed a gene conversion that involves the replacement of an A gamma sequence by a G gamma sequence in the promoter area of the A gamma gene. This event is similar to another gene conversion believed to be responsible for the very high homology between gamma-globin genes, suggesting that the promoter area of these genes is prone to this type of genetic rearrangement. Further analysis demonstrated that the chromosome bearing this gene conversion has a very high frequency among Bantu chromosomes and a very low or nil frequency in other haplotypes linked to the beta s gene. No correlation was found between the G gamma/A gamma ratio and the presence of the gene conversion among Bantu haplotype patients, thus excluding a portion of the gamma gene sequence in the determination of this ratio.
对一名纯合子为班图单倍型的镰状细胞贫血患者的γ-珠蛋白基因进行克隆和测序,结果显示发生了一次基因转换,该转换涉及在Aγ基因启动子区域中,一个Aγ序列被一个Gγ序列取代。这一事件类似于另一次被认为导致γ-珠蛋白基因间具有极高同源性的基因转换,表明这些基因的启动子区域易于发生此类基因重排。进一步分析表明,携带这种基因转换的染色体在班图染色体中出现频率极高,而在与βs基因连锁的其他单倍型中频率极低或几乎不存在。在班图单倍型患者中,未发现Gγ/Aγ比值与基因转换的存在之间存在相关性,因此在确定该比值时排除了γ基因序列的一部分。