The sulphydryl groups of sickle-cell haemoglobin

The sulphydryl groups of sickle-cell haemoglobin.

particularpH owing to the screening ofthe haematin iron atom from the influence of the various ionizing groups. On this basis a larger critical I would signify that a larger concentration of salt ions is required for complete screening, and hence that there is a stronger interaction between the iron and the ionizing groups in MetHb. In conclusion, although the mechanism may be different it may ...

Properties of sickle-cell haemoglobin.

Sickle-cell anaemia, sickle-cell thalassaemia, sickle-cell haemoglobin C disease, and asymptomatic haemoglobin C thalassaemia in one Ghanaian family.

A Ghanaian family is described in which a sickle-cell haemoglobin C man married to a sickle-cell thalassaemia woman produced 12 children (eight alive). Four children have sickle-cell anaemia, two sickle-cell haemoglobin C disease, one has sickle-cell thalassaemia, and one is asymptomatic haemoglobin C thalassaemia.It is emphasized that the contribution that adult sickle-cell disease patients ma...

Electrochemical modulation of sickle cell haemoglobin polymerisation.

Sickle cell haemoglobin (HbS) differs from normal haemoglobin by a single amino acid in its beta chain. This amino acid replacement, from glutamic acid to valine, causes polymerisation of proteins into defined long insoluble fibres with a typical diameter of 21.5 nm. The polymerisation is triggered by the formation of deoxyhaemoglobin (deoxyHb) from oxyhaemoglobin (oxyHb) in low oxygen partial ...

Foetal haemoglobin in sickle cell anemia.

Fetal hemoglobin (HbF) is the major genetic modulator of the hematologic and clinical features of sickle cell disease, an effect mediated by its exclusion from the sickle hemoglobin polymer. Fetal hemoglobin genes are genetically regulated, and the level of HbF and its distribution among sickle erythrocytes is highly variable. Some patients with sickle cell disease have exceptionally high level...