Hemoglobin Koelliker (α₂ minus 141 Arg β₂) in Favism

A New Hemoglobin Variant , Hemoglobin Nunobiki [ a 141 ( HC 3 ) Arg - + Cys ]

A new hemoglobin variant, hemoglobin (Hb) Nunobiki, was detected in a Japanese male with marginal erythrocytosis. The Hb Nunobiki component amounted to 13.1% of the total hemoglobin. Structural analysis of this variant established the substitution of a cysteine for an arginine at the carboxy terminus of the a-chain (a]141). The oxygen equilibrium curves of Hb Nunobiki revealed extremely high ox...

Favism.

Favism in London.

Favism is an acute haemolytic anaemia caused by an acquired allergy to a protein of the broad bean (Viciafava). Reports of the disease date from before the fifth century B.C. and in a good review of the literature Luisada (1941) points out that favism used to have a very wide distribution in the Mediterranean basin. It now occurs particularly in the Sardinians, who seem to have retained the mor...

Hemoglobin Willamette (β51Pro → Arg): Case Report and Literature Review

We report a case of hemoglobin (Hb) Willamette (β51 Pro → Arg) in the Hematology Department of a tertiary hospital in Fortaleza, Northeast of Brazil. A literature review of the cases described in health sciences databases using as a descriptor Hb Willamette was performed, revealing 12 reported cases, of which only one presented with anemia. Herein, we describe a case of a female 29 years old, w...

Characterization and oxygen binding properties of des-Arg human hemoglobin.

The role of chloride in the stabilization of the deoxy conformation of hemoglobin (Hb), the low oxygen affinity state, has been studied in order to identify the nature of this binding. Previous studies have shown that arginines 141alpha could be involved in the binding of this ion to the protein. Thus, des-Arg Hb, human hemoglobin modified by removal of the alpha-chain C-terminal residue Arg141...