Bard H, Widness J A, Ziegler E E, Gagnon C, Peri K G
Department of Pediatrics, University of Montreal, St. Justine's Hospital, Canada.
Pediatr Res. 1995 Mar;37(3):361-4. doi: 10.1203/00006450-199503000-00018.
The change in G gamma to A gamma ratio in relation to the switchover of fetal HB (HbF) to adult Hb (alpha 2 beta 2) synthesis has not been well defined. The gamma-globins of HbF (alpha 2 gamma 2) have either glycine (G gamma) or alanine (A gamma) at position 136. During fetal life the G gamma makes up 70% of the gamma-globins, although they are 40% of the small amounts of HbF in the adult. To further the understanding of this switchover, globin chain synthesis was determined sequentially in eight preterm and 20 full-term infants postnatally. To complete the study, single analysis was also carried out in eight term infants at birth and six preterm infants at the postconceptional age equivalent to term. Blood samples were incubated in an amino acid mixture containing 3H-leucine and subjected to reversed-phase liquid chromatography. The results demonstrated that the fetal proportions of G gamma to A gamma are rigidly controlled according to postconceptional age and not affected by postnatal age after preterm birth. During the early postconceptional age switchover from HbF to adult Hb synthesis, an equal repression of the G gamma and A gamma chains was found. However, based on the values obtained from the term infants, after a postconceptional age of 44 wk, the levels of G gamma to total gamma synthesis begin to decrease and become more variable.
胎儿血红蛋白(HbF)向成人血红蛋白(α2β2)合成转换过程中,Gγ与Aγ比例的变化尚未明确界定。HbF(α2γ2)的γ珠蛋白在第136位要么是甘氨酸(Gγ),要么是丙氨酸(Aγ)。在胎儿期,Gγ占γ珠蛋白的70%,尽管在成人中它们仅占少量HbF的40%。为了进一步了解这种转换,对8名早产儿和20名足月儿出生后的珠蛋白链合成进行了连续测定。为完成研究,还对8名足月儿出生时和6名孕龄相当于足月儿的早产儿进行了单次分析。将血样在含有3H-亮氨酸的氨基酸混合物中孵育,然后进行反相液相色谱分析。结果表明,Gγ与Aγ的胎儿比例严格按照孕龄控制,不受早产产后年龄的影响。在孕龄早期从HbF向成人血红蛋白合成转换过程中,发现Gγ和Aγ链受到同等程度的抑制。然而,根据足月儿获得的值,孕龄44周后,Gγ与总γ合成的水平开始下降且变得更具变异性。