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Sickle-Cell Anemia: Molecular and Cellular Bases of Therapeutic Approaches

1978; Massachusetts Medical Society; Volume: 299; Issue: 14 Linguagem: Inglês

10.1056/nejm197810052991405

1533-4406

Jurrien Dean, Alan N. Schechter,

Neonatal Health and Biochemistry

THE term "molecular disease" was coined almost 30 years ago by Linus Pauling and his associates to describe their analysis of the abnormality in sickle-cell anemia.1 Since that time tremendous progress has been made in the understanding of the structure and function of this mutant hemoglobin molecule and the conditions under which it aggregates when deoxygenated to form a gel. Indeed, in the last decade, particularly in the last few years, there has been an explosive growth in knowledge of the structure of the gel of sickle hemoglobin and the mechanism of its formation. The exact relations among gelation, sickling . . .