Lapouméroulie C, Dunda O, Ducrocq R, Trabuchet G, Mony-Lobé M, Bodo J M, Carnevale P, Labie D, Elion J, Krishnamoorthy R
INSERM U 120, Hôpital Robert Debré, Paris, France.
Hum Genet. 1992 May;89(3):333-7. doi: 10.1007/BF00220553.
The sickle cell mutation (beta s) arose as at least three independent events in Africa and once in Asia, being termed the Senegal, Benin, Bantu and Indian types respectively. An investigation in Cameroon was carried out to determine whether the atypical sickle genes observed in the neighboring countries are the result of recombination or the presence of a sickle cell mutation of a different genetic origin. It was conducted on 40 homozygous SS patients followed at the Blood Transfusion Center in the capital city of Yaoundé. On 80 beta s chromosomes, 13 exhibited a novel polymorphic pattern that was observed three times in the homozygous state. This chromosome contains an A gamma T gene. The restriction fragment length polymorphism haplotype is different from all the other beta s chromosomes in both the 5' and 3' regions, but has previously been reported in sporadic cases. The (AT)8(T)5 sequence in the -500 region of the beta gene is specific and different from that of the Senegal, Benin, Bantu or Indian beta s genes. All the carriers of this specific chromosome belong to the Eton ethnic group and originate from the Sanaga river valley. This observation strongly argues for yet another independent origin of the sickle cell mutation in Africa, here referred to as the "Cameroon type". The Benin haplotype and a Benin/Bantu recombinant haplotype have been observed in the other studied populations: Ewondo, Bamiléké, Bassa, Yambassa and Boulou.
镰状细胞突变(βs)在非洲至少作为三个独立事件出现,在亚洲出现过一次,分别被称为塞内加尔型、贝宁型、班图型和印度型。在喀麦隆开展了一项调查,以确定在邻国观察到的非典型镰状基因是重组的结果还是不同遗传起源的镰状细胞突变的存在。该调查针对在首都雅温得输血中心随访的40名纯合SS患者进行。在80条βs染色体上,有13条呈现出一种新的多态性模式,在纯合状态下被观察到三次。这条染色体包含一个AγT基因。限制性片段长度多态性单倍型在5'和3'区域均与所有其他βs染色体不同,但此前在散发病例中已有报道。β基因-500区域的(AT)8(T)5序列是特异的,与塞内加尔、贝宁、班图或印度的βs基因不同。这条特定染色体的所有携带者都属于埃顿族,来自萨纳加河谷。这一观察结果有力地证明了非洲镰状细胞突变的另一个独立起源,在此称为“喀麦隆型”。在其他研究人群:埃翁多族、巴米累克族、巴萨族、扬巴萨族和布卢族中观察到了贝宁单倍型和贝宁/班图重组单倍型。