This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables.
The material presented is divided into eight chapters. The first three, authored by Dr. Murayama, discuss the chemical and three-dimensional structure of human hemoglobin, the bioenergetics of sickling, and Dr. Murayama's hypotheses explaining the mechanism of the sickling phenomenon. This material is stated in a clear and concise fashion. After discussing the primary, secondary, tertiary, and quarternary structure of the hemoglobin molecule, Dr. Murayama reviews his published experiments and uses them as a basis for his hypothesis of the sickling phenomenon, including the need for a cofactor. Not everybody agrees with Dr. Murayama's ideas, but they are well presented here.
Dr. Nalbandian follows these introductory chapters with a detailed discussion of his ideas on sickle cell disease, starting with intrauterine erythropoeisis, and then discusses the Murayama (dithionate-urea) test, an automated mass screening program for sickle cell disease, and the theoretical and clinical practical use of urea in sickle cell disease.
Kraus AP. Sickle Cell Hemoglobin: Molecule to Man. JAMA. 1974;227(7):805–806. doi:10.1001/jama.1974.03230200063029
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: