Martínez G, Lima F, Colombo B
Biochim Biophys Acta. 1977 Mar 28;491(1):1-6. doi: 10.1016/0005-2795(77)90034-4.
Haemoglobin J Guantanamo (alpha 2 beta 2 128 (H6) Ala replaced by Asp) was found during a screening from abnormal haemoglobins in three members of a Cuban family, of negro ancestry. The substitution in this variant is located at the alpha 1 beta 1 contact. This explains the slight instability and the mild haemolytic anaemia and morphological abnormalities found in the carriers of this variant. The instability of haemoglobin J Guantanamo indicates that the presence of Asp at the position beta-128 (H6) weakens the alpha 1 beta 1 contact.
