216k views
5 votes
Do patients with α-thalassemia due to a four gene deletion have any α-globin?

1 Answer

3 votes

Final answer:

No, patients with α-thalassemia due to a four-gene deletion do not have any α-globin.

Step-by-step explanation:

In individuals with α-thalassemia, a genetic disorder affecting the synthesis of hemoglobin, the α-globin gene cluster is affected. The severity of the condition depends on the number of affected α-globin genes. Normally, there are four α-globin genes (two on each chromosome 16), and a four-gene deletion implies the loss of all four α-globin genes. As a result, individuals with this condition do not produce functional α-globin chains, which are essential components of hemoglobin.

Hemoglobin is composed of two α-globin and two β-globin chains. In α-thalassemia with a four-gene deletion, the absence of α-globin chains disrupts the normal formation of hemoglobin.

This leads to an imbalance in globin chain production, causing ineffective erythropoiesis and red blood cell abnormalities. The lack of α-globin chains also contributes to the formation of insoluble aggregates within red blood cells, leading to their premature destruction and anemia.

In summary, individuals with α-thalassemia due to a four-gene deletion do not have any functional α-globin chains. This absence of α-globin disrupts the synthesis of normal hemoglobin, resulting in the characteristic features of α-thalassemia, including anemia and abnormal red blood cell morphology.

User BenTobin
by
8.5k points