Iron Metabolism and Etiology of Iron-Storage Diseases: An Interpretive Formulation

• Published in: JAMA : the journal of the American Medical Association Vol. 189; no. 1; pp. 45 - 53
• Main Author: Mendel, Gerald A
• Format: Journal Article
• Language: English
• Published: United States American Medical Association 06-07-1964
• Subjects: Absorption; Anemia; Genetics, Medical; Hemochromatosis; Hemosiderosis; Humans; Iron - metabolism; Metabolic Diseases; Old Medline; HEMOSIDEROSIS; ABSORPTION; ANEMIA; METABOLIC DISEASES; GENETICS, HUMAN; IRON METABOLISM; HEMOCHROMATOSIS
• ISSN: 0098-7484; 1538-3598
• DOI: 10.1001/jama.1964.03070010051011

ALTHOUGH iron is present in only small quantities in the human organism, iron-containing compounds are of vital biological importance. The interest of the physician, biochemist, and physiologist in iron metabolism stems from the role played by this element in oxygen transport between cells, in the utilization of oxygen within the cell, in hemoglobin synthesis and erythropoiesis, and in the disease states caused by iron-lack and iron overload.In contrast to the prevalence of iron deficiency, the diseases associated with iron overload are rare. In spite of this infrequency, their pathogenesis has been investigated with vigor for many years. Numerous problems remain unsolved and there are many areas of controversy. Thus, the view that hemochromatosis is an inherited inborn error of metabolism continues to be challenged, and there is no unanimity of opinion on the relationship between iron accumulation and tissue damage.This paper is not intended as a comprehensive review,