Haemoglobin : model strategies for molecular disease

Abstract

Haemoglobin occupies a special position among the many proteins of man and other animals. It is among the most abundant of proteins and one of the best studied. Traditionally, haemoglobin has occupied the leading edge in the development of our understanding on the structure, function and biosynthesis of proteins and more recently in molecular biology and medicine. Approximately fifteen years since a-thalassaemia was documented as the first molecular disease, we take this opportunity to review some of the most salient features of haemoglobin molecular biology and relate this body of knowledge to the development of strategies that contribute in understanding other problems in molecular medicine and improve patient care. Haemoglobin R & D programmes offer tremendous opportunities to enhance the education and training of young scientists in developing countries and help to develop high quality services. The term molecular disease is relatively new in medical nomenclature. It refers to the study of a range of conditions which are due to abnormalities in the sequence or organisation of the nuclear or mitochondrial nucleic acids. These encompass genetics, oncology and infectious disease, typically viral infections.