Hemoglobin dhonburi α2β2 126 (H4) Val → Gly: A new unstable β variant producing a β‐thalassemia intermedia phenotype in association with β°‐Thalassemia

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Abstract
While investigating the mechanism of a β‐thalassemia intermedia phenotype in a 34 year old Thai male, a new Hb variant β 126 Val → Gly named Hb Dhonburi was discovered. Genetic and structural studies revealed the existence of a β°‐thalassemia genotype in association with the β variant. The new variant is unstable but exhibits normal oxygen binding properties. Hb Dhonburi was also discovered in the mother of the propositus in association with Hb E.