Hemoglobin Rainier (β145 Tyrosine → Histidine): Alkali-Resistant Hemoglobin with Increased Oxygen Affinity

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Science  16 Feb 1968:
Vol. 159, Issue 3816, pp. 741-743
DOI: 10.1126/science.159.3816.741


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.