1.Normally, hemoglobin is made up of four globin chains, each bound to a heme group.

况下，白由4条珠白链组成，每条珠白链都与一个素基团结合。

2.These four globin chains combine in different ways to give rise to different kinds of hemoglobin.

这四种珠白链以不同的方式结合，形成不同类型的白。

3.A hemoglobin tetramer with two alpha-globin and two mutated beta-globin proteins is called sickle hemoglobin, or HbS.

由两条α-珠白与两条变异β-珠白构成的白四聚体，称作镰状白，或HbS。

4.Now hemoglobin is made up of hemes and globins.

白由素与珠白构成。

5.Specifically, the beta-globin chains end up misshapen, and this is because of a mutation in the beta-globin gene or HBB gene.

确切来说，（该病中）β-珠白链发生变形，而这是由β-珠白基因（HBB基因）的突变造成的。

6.Fetal hemoglobin is made up of two alpha- and two gamma-globin chains, so it doesn't include the mutated beta-globins.

HbF由两条α-和两条γ-珠白链构成，不包括突变的β-珠白。

7.Remember that the globin in your hemoglobin is a protein — and when proteins bind to stuff, they tend to change shape.

记住白中的珠白是一种白质，当白质和其他物质结合时它们就会改变形状。

8.Sickle cell is an autosomal recessive disease, so a mutation in both copies of the beta-globin gene is needed to get the disease.

镰贫是一种染色体隐性遗传病，因此需要两个β-珠白基因序列均突变才能获得该病。

9.This oxygen is bound to hemoglobin, which consists of an iron-containing pigment called heme and a protein part called globin.

这些氧气与白结合，白由叫做素的含铁的色素和叫做珠白的白质部分组成。

10.Hemoglobin A (or HbA) - made up of two alpha-globin and two beta-globin peptide chains - is the primary hemoglobin affected in sickle cell.

白A（HbA）由两条α-及两条β-珠白肽链构成，是镰贫中主要被影响的白。

11.All right, as a quick recap: alpha thalassemia is an autosomal recessive disorder caused by deletion of alpha globin genes on chromosome 16.

得！快速回顾一下：α地中海贫是一种染色体隐性遗传病，由16号染色体上的α珠白基因缺陷引起。

12.Another preventative medicine is hydroxyurea - which works by increasing the amount of gamma-globin - which results in more fetal hemoglobin, or HbF.

另一种预防药物是羟基脲；它通过增加γ-珠白，以产生更多的胎儿白（HbF）。

13.Almost always, the sickle cell mutation is a non-conservative missense mutation that results in the sixth amino acid of beta-globin being valine instead of glutamic acid.

镰状细胞突变几乎总是由于非保守区错义突变，导致β-珠白的第6个氨基酸为缬氨酸，而非谷氨酸。

14.Free hemoglobin in the plasma is bound by a molecule called haptoglobin and gets recycled, which is why a low haptoglobin level is a sign of intravascular hemolysis.

浆游离白与结合珠白结合，并被循环利用，这也是为何低结合珠白水平是管内溶的标志之一。

15.As a quick recap: sickle cell disease is an autosomal recessive genetic disease where the beta-globin subunit of hemoglobin is misshapen, which causes red blood cells to sickle when deoxygenated.

好！简要回顾一下：镰状细胞病，是染色体隐性遗传病，其中白的β-珠白发生突变，脱氧况下会导致细胞镰变。