Hemoglobin Rainier (β 145 Tyrosine → Histidine): Alkali-Resistant Hemoglobin with Increased Oxygen Affinity

Abstract
Hemoglobin Rainier, a new hemoglobin variant associated with erythrocytosis, was found in six members of a Caucasiant family. Structurally, it represents substitution of histidine for the invariant residute H23 tyrosine in the β-hemoglobin polypeptide chain (β145 tyrosine → histidine). Hemoglobin Rainier is the first example of a single amino acid substitution in adult human hemoglobin, causing increased resistance to alkali denaturation.