Elongation of α globin chains, does excessive amino acids and helices relate to clinical manifestation?

摘要

Haemoglobin variants in which a disorder results in chain elongation are unusual. For α globin chain, the elongation of haemoglobin is believed to relate to the clinical manifestations such as those in hemoglobin H diseases. Although the primary structures of Hb disorders with elongated α globin chains are well-known the secondary structure of them is not well documented. The study on the tertiary structures of the elongated part in those Hb can help explain more in the pathogenesis of the disorders is needed. Here, the author performs a bioinformatic analysis for nanohematology to study the secondary structures of three well described Hb disorders, Hb Pak Num Po, Hb Pakse and Hb Constant Spring with elongated α globin chains. According to the analysis, the trend of increase aberration relating with increased excessive amino acids and excessive helical residues are noted. However, generalization to other Hb disorders, than these 3 disorders, needs further experimental studies..