What is the fundamental defect that results in beta-thalassemia major?
A) The spleen prematurely destroys the precipitate-carrying cells.
B) A severe uncoupling of a- and b-chain synthesis occurs.
C) All four beta-forming genes are defective.
D) Hemoglobin H (HbH) develops when three genes are defective.

Question

Quizplus · Accepted Answer

The fundamental defect in beta-thalassemia is the uncoupling of a- and b-chain synthesis.b-Chain production is moderately depressed in the heterozygous form-beta-thalassemia minor,and severely depressed in the homozygous form-beta-thalassemia major (also called Cooley anemia).The defects described in the other options are not related to beta-thalassemia major.

What Is the Fundamental Defect That Results in Beta-Thalassemia Major