Archibald Edward Garrod, “Inborn Errors of Metabolism”, 1908 was a nonfiction science book. Among the many “inborn errors of metabolism” described by Archibald Edward Garrod was “Sickle Cell Anemia”. However, today we would not classify “Sickle Cell Anemia” as aa “inborn error of metabolism”. That having been said, I will discuss “Sickle Cell Anemia” which is a disorder of the “hemoglobin” within “red blood cells”. The blood normally contains 20-12 grams of hemoglobin per deciliter. The chemical formula for hemoglobin is C2952H4664O832N812S8Fe4; so the protein is technically beyond Archibald Edward Garrod in 1908 with 9272 atoms in hemoglobin. Shown in the diagram below, molecular oxygen O=O binds to the hemoglobin at the iron Fe(II) histidine amino-acid. Archibald Edward Garrod was able to diagnosis the disorder due to shape of the RBC changing from “biconcave” to “sickle”. The RBC cytoplasm dry-weight is 90% hemoglobin, this explains the observed shape change in the RBC. Historically signifant is: Linus Pauling, Harvey A. Itano. S. J. Singer, and Ibert C. W. Wells, “Sickle Cell Anemia: A Molecular Disease”, Science, 1949, volume 110, page 543-548 in the research paper the differences between normal and sickle hemoglobin are presented. Linus Pauling had been researching hemoglobin since 1935 and is presenting results in the 1949 paper.
